2,2-dimethylbutyric acid profile

2,2-dimethylbutyric acid: structure given in first source; plasma metabolite of simvastatin [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

2,2-dimethylbutyric acid : A branched-chain fatty acid and metabolite of the lactone prodrug simvastatin, whose sodium salt is potentially useful for the treatment of thalassaemias and haemoglobinopathies. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

ID Source	ID
PubMed CID	11684
CHEMBL ID	286013
CHEBI ID	38649
SCHEMBL ID	47533
MeSH ID	M0201541

Synonym
ec 209-865-0
nsc 16045
ay606cn05o ,
einecs 209-865-0
unii-ay606cn05o
MLS002174247
smr001261422
butanoic acid, 2,2-dimethyl-
butyric acid, 2,2-dimethyl-
alpha,alpha-dimethylbutanoic acid
CHEBI:38649 ,
alpha,alpha-dimethylbutyric acid
.alpha.,.alpha.-dimethylbutyric acid
2,2-dimethylbutanoic acid
butanoic acid,2-dimethyl-
nsc-16045
neohexanoic acid
2,2-dimethylbutyric acid
.alpha.,.alpha.-dimethylbutanoic acid
nsc16045
595-37-9
NCGC00090948-01
2,2-dimethyl butanoic acid
inchi=1/c6h12o2/c1-4-6(2,3)5(7)8/h4h2,1-3h3,(h,7,8
2,2-dimethylbutyric acid, 96%
LMFA01020078
2,2-dimethyl-butanoic acid
dimethyl ethyl acetic acid
CHEMBL286013
dimebutic acid
nsc-741804
2,2-dimethyl-butyric acid
FT-0653122
D2429
NCGC00090948-02
AKOS009158866
EN300-64847
HMS3039B09
cas-595-37-9
dtxsid0032811 ,
dtxcid8012811
tox21_200740
NCGC00258294-01
FT-0609269
dimebutic acid [inn]
SCHEMBL47533
W-105316
2,2-dimethylbutyrate
1219804-04-2
2,2-dimethylbutyric-d11 acid
mfcd00004200
CS-W016597
DS-14471
Q27117928
2.2-dimethylbutyric acid
2,2-dimethylbutanoicacid-d6
2,2-dimethylbutanoicacid
bdbm50566497
Z995036360

Excerpt	Reference	Relevance
" Healthy adult human subjects were treated with a novel short chain fatty acids (SCFA) derivative, sodium 2,2 dimethylbutyrate (SDMB), or placebo, with 1 of 4 single dose levels (2, 5, 10, and 20 mg/kg) or daily doses (5, 10, or 15 mg/kg) over 14 days, and monitored for adverse clinical and laboratory events, drug levels, reticulocytes, and HbF assays."	( Evaluation of safety and pharmacokinetics of sodium 2,2 dimethylbutyrate, a novel short chain fatty acid derivative, in a phase 1, double-blind, placebo-controlled, single-dose, and repeat-dose studies in healthy volunteers. Berenson, RJ; Boosalis, MS; Case, S; Faller, DV; Keefer, JR; Perrine, SP; Wallis, WJ; Wargin, WA; Welch, WC, 2011)	0.37
" In conclusion, the no-observed adverse effect level for FOB and general toxicity was 200 mg/kg following gavage administration of ST-20 for up to 15 consecutive days."	( Short-term toxicity study of ST-20 (NSC-741804) by oral gavage in Sprague-Dawley rats. Contos, DA; Hawk, MA; Johnson, JD; Peggins, JO; Perrine, SP; Ritchie, GD; Ryan, MJ; Terse, PS; Tomaszewski, JE; Vasconcelos, DY, 2011)	0.37
" The most common adverse events in the HQK-1001 group, usually graded as mild or moderate, consisted of nausea, headache, vomiting, abdominal pain, and fatigue."	( A double-blind, placebo-controlled phase II study of the efficacy and safety of 2,2-dimethylbutyrate (HQK-1001), an oral fetal globin inducer, in sickle cell disease. Abboud, MR; El Beshlawy, A; Ghalie, RG; Haynes, J; Inati, A; Kutlar, A; Manwani, D; Reid, ME; Sharon, B; Smith, W; Taher, AT; Ward, R, 2014)	0.4

Excerpt	Reference	Relevance
" The terminal half-life ranged from 9 to 15 hours."	( Evaluation of safety and pharmacokinetics of sodium 2,2 dimethylbutyrate, a novel short chain fatty acid derivative, in a phase 1, double-blind, placebo-controlled, single-dose, and repeat-dose studies in healthy volunteers. Berenson, RJ; Boosalis, MS; Case, S; Faller, DV; Keefer, JR; Perrine, SP; Wallis, WJ; Wargin, WA; Welch, WC, 2011)	0.37

Excerpt	Reference	Relevance
" Sodium 2,2 dimethylbutyrate (HQK-1001), an orally bioavailable short-chain fatty acid derivative, induces γ-globin expression experimentally and is well-tolerated in normal subjects."	( A randomized phase I/II trial of HQK-1001, an oral fetal globin gene inducer, in β-thalassaemia intermedia and HbE/β-thalassaemia. Berenson, R; Boosalis, M; Chaneim, N; Fucharoen, S; Inati, A; Koussa, S; Perrine, SP; Siritanaratku, N; Taher, A; Thein, SL; Wargin, WC, 2013)	0.39

Excerpt	Relevance	Reference
" A fetal globin-inducing therapeutic with convenient oral dosing would be an advance for these classic molecular diseases."	( Evaluation of safety and pharmacokinetics of sodium 2,2 dimethylbutyrate, a novel short chain fatty acid derivative, in a phase 1, double-blind, placebo-controlled, single-dose, and repeat-dose studies in healthy volunteers. Berenson, RJ; Boosalis, MS; Case, S; Faller, DV; Keefer, JR; Perrine, SP; Wallis, WJ; Wargin, WA; Welch, WC, 2011)	0.37
" Further investigation of HQK-1001 with longer dosing to definitively evaluate its haematological potential appears warranted."	( A randomized phase I/II trial of HQK-1001, an oral fetal globin gene inducer, in β-thalassaemia intermedia and HbE/β-thalassaemia. Berenson, R; Boosalis, M; Chaneim, N; Fucharoen, S; Inati, A; Koussa, S; Perrine, SP; Siritanaratku, N; Taher, A; Thein, SL; Wargin, WC, 2013)	0.39

Role	Description
metabolite	Any intermediate or product resulting from metabolism. The term 'metabolite' subsumes the classes commonly known as primary and secondary metabolites.
[role information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Class	Description
dimethylbutyric acid	Any compound comprising a butyric acid skeleton carrying two methyl components.
[compound class information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Protein	Taxonomy	Measurement	Average (µ)	Min (ref.)	Avg (ref.)	Max (ref.)	Bioassay(s)
chaperonin-containing TCP-1 beta subunit homolog	Homo sapiens (human)	Potency	50.1187	3.9811	27.7649	39.8107	AID504842
GLI family zinc finger 3	Homo sapiens (human)	Potency	60.3233	0.0007	14.5928	83.7951	AID1259369
chromobox protein homolog 1	Homo sapiens (human)	Potency	89.1251	0.0060	26.1688	89.1251	AID540317
potassium voltage-gated channel subfamily H member 2 isoform d	Homo sapiens (human)	Potency	12.5893	0.0178	9.6374	44.6684	AID588834
geminin	Homo sapiens (human)	Potency	0.1490	0.0046	11.3741	33.4983	AID624296; AID624297
Nuclear receptor ROR-gamma	Homo sapiens (human)	Potency	3.7578	0.0266	22.4482	66.8242	AID651802
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Protein	Taxonomy	Measurement	Average	Min (ref.)	Avg (ref.)	Max (ref.)	Bioassay(s)
Cytochrome P450 1A2	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0001	1.7740	10.0000	AID1750639; AID1750649
Cytochrome P450 3A4	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0001	1.7536	10.0000	AID1750645; AID1750646; AID1750647; AID1750655; AID1750656; AID1750657; AID1750658
Cytochrome P450 2C8	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0008	1.8848	7.9000	AID1750651
Cytochrome P450 2D6	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0000	2.0151	10.0000	AID1750644; AID1750654
Cytochrome P450 2C9	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0000	2.8005	10.0000	AID1750642; AID1750652
Cytochrome P450 2B6	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0011	3.4186	10.0000	AID1750640; AID1750650
Cytochrome P450 3A5	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0033	0.7073	6.2000	AID1750648
Cytochrome P450 2C19	Homo sapiens (human)	IC50 (µMol)	10,000.0000	0.0000	2.3983	10.0000	AID1750643; AID1750653
Solute carrier family 22 member 6	Homo sapiens (human)	IC50 (µMol)	3,862.0000	0.2700	4.5306	9.9000	AID1750663
Solute carrier family 22 member 8	Homo sapiens (human)	IC50 (µMol)	3,511.0000	4.9300	7.3900	9.9200	AID1750664
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Process	via Protein(s)	Taxonomy
steroid catabolic process	Cytochrome P450 1A2	Homo sapiens (human)
porphyrin-containing compound metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
cholesterol metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
toxin biosynthetic process	Cytochrome P450 1A2	Homo sapiens (human)
post-embryonic development	Cytochrome P450 1A2	Homo sapiens (human)
alkaloid metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
regulation of gene expression	Cytochrome P450 1A2	Homo sapiens (human)
monoterpenoid metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
dibenzo-p-dioxin metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
epoxygenase P450 pathway	Cytochrome P450 1A2	Homo sapiens (human)
lung development	Cytochrome P450 1A2	Homo sapiens (human)
methylation	Cytochrome P450 1A2	Homo sapiens (human)
monocarboxylic acid metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 1A2	Homo sapiens (human)
retinol metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
long-chain fatty acid biosynthetic process	Cytochrome P450 1A2	Homo sapiens (human)
cellular respiration	Cytochrome P450 1A2	Homo sapiens (human)
aflatoxin metabolic process	Cytochrome P450 1A2	Homo sapiens (human)
hydrogen peroxide biosynthetic process	Cytochrome P450 1A2	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 1A2	Homo sapiens (human)
cellular response to cadmium ion	Cytochrome P450 1A2	Homo sapiens (human)
omega-hydroxylase P450 pathway	Cytochrome P450 1A2	Homo sapiens (human)
lipid hydroxylation	Cytochrome P450 3A4	Homo sapiens (human)
lipid metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
steroid catabolic process	Cytochrome P450 3A4	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
cholesterol metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
androgen metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
alkaloid catabolic process	Cytochrome P450 3A4	Homo sapiens (human)
monoterpenoid metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
calcitriol biosynthetic process from calciol	Cytochrome P450 3A4	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 3A4	Homo sapiens (human)
vitamin D metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
vitamin D catabolic process	Cytochrome P450 3A4	Homo sapiens (human)
retinol metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
retinoic acid metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
long-chain fatty acid biosynthetic process	Cytochrome P450 3A4	Homo sapiens (human)
aflatoxin metabolic process	Cytochrome P450 3A4	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 3A4	Homo sapiens (human)
lipid hydroxylation	Cytochrome P450 2C8	Homo sapiens (human)
organic acid metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
epoxygenase P450 pathway	Cytochrome P450 2C8	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 2C8	Homo sapiens (human)
retinol metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
retinoic acid metabolic process	Cytochrome P450 2C8	Homo sapiens (human)
long-chain fatty acid biosynthetic process	Cytochrome P450 2C8	Homo sapiens (human)
icosanoid biosynthetic process	Cytochrome P450 2C8	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 2C8	Homo sapiens (human)
omega-hydroxylase P450 pathway	Cytochrome P450 2C8	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
cholesterol metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
coumarin metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
alkaloid metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
alkaloid catabolic process	Cytochrome P450 2D6	Homo sapiens (human)
monoterpenoid metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
isoquinoline alkaloid metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 2D6	Homo sapiens (human)
retinol metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
long-chain fatty acid biosynthetic process	Cytochrome P450 2D6	Homo sapiens (human)
negative regulation of binding	Cytochrome P450 2D6	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 2D6	Homo sapiens (human)
negative regulation of cellular organofluorine metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
arachidonic acid metabolic process	Cytochrome P450 2D6	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
cholesterol metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
monoterpenoid metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
epoxygenase P450 pathway	Cytochrome P450 2C9	Homo sapiens (human)
urea metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
monocarboxylic acid metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 2C9	Homo sapiens (human)
long-chain fatty acid biosynthetic process	Cytochrome P450 2C9	Homo sapiens (human)
amide metabolic process	Cytochrome P450 2C9	Homo sapiens (human)
icosanoid biosynthetic process	Cytochrome P450 2C9	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 2C9	Homo sapiens (human)
omega-hydroxylase P450 pathway	Cytochrome P450 2C9	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 2B6	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 2B6	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 2B6	Homo sapiens (human)
cellular ketone metabolic process	Cytochrome P450 2B6	Homo sapiens (human)
epoxygenase P450 pathway	Cytochrome P450 2B6	Homo sapiens (human)
lipid hydroxylation	Cytochrome P450 3A5	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
estrogen metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
alkaloid catabolic process	Cytochrome P450 3A5	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 3A5	Homo sapiens (human)
retinol metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
retinoic acid metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
aflatoxin metabolic process	Cytochrome P450 3A5	Homo sapiens (human)
oxidative demethylation	Cytochrome P450 3A5	Homo sapiens (human)
long-chain fatty acid metabolic process	Cytochrome P450 2C19	Homo sapiens (human)
xenobiotic metabolic process	Cytochrome P450 2C19	Homo sapiens (human)
steroid metabolic process	Cytochrome P450 2C19	Homo sapiens (human)
monoterpenoid metabolic process	Cytochrome P450 2C19	Homo sapiens (human)
epoxygenase P450 pathway	Cytochrome P450 2C19	Homo sapiens (human)
xenobiotic catabolic process	Cytochrome P450 2C19	Homo sapiens (human)
omega-hydroxylase P450 pathway	Cytochrome P450 2C19	Homo sapiens (human)
negative regulation of transcription by RNA polymerase II	Nuclear receptor ROR-gamma	Homo sapiens (human)
xenobiotic metabolic process	Nuclear receptor ROR-gamma	Homo sapiens (human)
regulation of glucose metabolic process	Nuclear receptor ROR-gamma	Homo sapiens (human)
regulation of steroid metabolic process	Nuclear receptor ROR-gamma	Homo sapiens (human)
intracellular receptor signaling pathway	Nuclear receptor ROR-gamma	Homo sapiens (human)
circadian regulation of gene expression	Nuclear receptor ROR-gamma	Homo sapiens (human)
cellular response to sterol	Nuclear receptor ROR-gamma	Homo sapiens (human)
positive regulation of circadian rhythm	Nuclear receptor ROR-gamma	Homo sapiens (human)
regulation of fat cell differentiation	Nuclear receptor ROR-gamma	Homo sapiens (human)
positive regulation of DNA-templated transcription	Nuclear receptor ROR-gamma	Homo sapiens (human)
adipose tissue development	Nuclear receptor ROR-gamma	Homo sapiens (human)
T-helper 17 cell differentiation	Nuclear receptor ROR-gamma	Homo sapiens (human)
regulation of transcription by RNA polymerase II	Nuclear receptor ROR-gamma	Homo sapiens (human)
monoatomic anion transport	Solute carrier family 22 member 6	Homo sapiens (human)
response to organic cyclic compound	Solute carrier family 22 member 6	Homo sapiens (human)
inorganic anion transport	Solute carrier family 22 member 6	Homo sapiens (human)
organic anion transport	Solute carrier family 22 member 6	Homo sapiens (human)
prostaglandin transport	Solute carrier family 22 member 6	Homo sapiens (human)
alpha-ketoglutarate transport	Solute carrier family 22 member 6	Homo sapiens (human)
xenobiotic transport	Solute carrier family 22 member 6	Homo sapiens (human)
sodium-independent organic anion transport	Solute carrier family 22 member 6	Homo sapiens (human)
transmembrane transport	Solute carrier family 22 member 6	Homo sapiens (human)
metanephric proximal tubule development	Solute carrier family 22 member 6	Homo sapiens (human)
renal tubular secretion	Solute carrier family 22 member 6	Homo sapiens (human)
monoatomic ion transport	Solute carrier family 22 member 8	Homo sapiens (human)
response to toxic substance	Solute carrier family 22 member 8	Homo sapiens (human)
inorganic anion transport	Solute carrier family 22 member 8	Homo sapiens (human)
prostaglandin transport	Solute carrier family 22 member 8	Homo sapiens (human)
xenobiotic transport	Solute carrier family 22 member 8	Homo sapiens (human)
transmembrane transport	Solute carrier family 22 member 8	Homo sapiens (human)
transport across blood-brain barrier	Solute carrier family 22 member 8	Homo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Process	via Protein(s)	Taxonomy
monooxygenase activity	Cytochrome P450 1A2	Homo sapiens (human)
iron ion binding	Cytochrome P450 1A2	Homo sapiens (human)
protein binding	Cytochrome P450 1A2	Homo sapiens (human)
electron transfer activity	Cytochrome P450 1A2	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 1A2	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 1A2	Homo sapiens (human)
enzyme binding	Cytochrome P450 1A2	Homo sapiens (human)
heme binding	Cytochrome P450 1A2	Homo sapiens (human)
demethylase activity	Cytochrome P450 1A2	Homo sapiens (human)
caffeine oxidase activity	Cytochrome P450 1A2	Homo sapiens (human)
aromatase activity	Cytochrome P450 1A2	Homo sapiens (human)
estrogen 16-alpha-hydroxylase activity	Cytochrome P450 1A2	Homo sapiens (human)
estrogen 2-hydroxylase activity	Cytochrome P450 1A2	Homo sapiens (human)
hydroperoxy icosatetraenoate dehydratase activity	Cytochrome P450 1A2	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 3A4	Homo sapiens (human)
steroid binding	Cytochrome P450 3A4	Homo sapiens (human)
iron ion binding	Cytochrome P450 3A4	Homo sapiens (human)
protein binding	Cytochrome P450 3A4	Homo sapiens (human)
steroid hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
retinoic acid 4-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 3A4	Homo sapiens (human)
oxygen binding	Cytochrome P450 3A4	Homo sapiens (human)
enzyme binding	Cytochrome P450 3A4	Homo sapiens (human)
heme binding	Cytochrome P450 3A4	Homo sapiens (human)
vitamin D3 25-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
caffeine oxidase activity	Cytochrome P450 3A4	Homo sapiens (human)
quinine 3-monooxygenase activity	Cytochrome P450 3A4	Homo sapiens (human)
testosterone 6-beta-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
1-alpha,25-dihydroxyvitamin D3 23-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
anandamide 8,9 epoxidase activity	Cytochrome P450 3A4	Homo sapiens (human)
anandamide 11,12 epoxidase activity	Cytochrome P450 3A4	Homo sapiens (human)
anandamide 14,15 epoxidase activity	Cytochrome P450 3A4	Homo sapiens (human)
aromatase activity	Cytochrome P450 3A4	Homo sapiens (human)
vitamin D 24-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
estrogen 16-alpha-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
estrogen 2-hydroxylase activity	Cytochrome P450 3A4	Homo sapiens (human)
1,8-cineole 2-exo-monooxygenase activity	Cytochrome P450 3A4	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 2C8	Homo sapiens (human)
iron ion binding	Cytochrome P450 2C8	Homo sapiens (human)
protein binding	Cytochrome P450 2C8	Homo sapiens (human)
arachidonic acid epoxygenase activity	Cytochrome P450 2C8	Homo sapiens (human)
retinoic acid 4-hydroxylase activity	Cytochrome P450 2C8	Homo sapiens (human)
caffeine oxidase activity	Cytochrome P450 2C8	Homo sapiens (human)
aromatase activity	Cytochrome P450 2C8	Homo sapiens (human)
estrogen 16-alpha-hydroxylase activity	Cytochrome P450 2C8	Homo sapiens (human)
heme binding	Cytochrome P450 2C8	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 2C8	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 2D6	Homo sapiens (human)
iron ion binding	Cytochrome P450 2D6	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 2D6	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 2D6	Homo sapiens (human)
heme binding	Cytochrome P450 2D6	Homo sapiens (human)
anandamide 8,9 epoxidase activity	Cytochrome P450 2D6	Homo sapiens (human)
anandamide 11,12 epoxidase activity	Cytochrome P450 2D6	Homo sapiens (human)
anandamide 14,15 epoxidase activity	Cytochrome P450 2D6	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
iron ion binding	Cytochrome P450 2C9	Homo sapiens (human)
arachidonic acid epoxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
steroid hydroxylase activity	Cytochrome P450 2C9	Homo sapiens (human)
arachidonic acid 14,15-epoxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
arachidonic acid 11,12-epoxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 2C9	Homo sapiens (human)
(S)-limonene 6-monooxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
(S)-limonene 7-monooxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
caffeine oxidase activity	Cytochrome P450 2C9	Homo sapiens (human)
(R)-limonene 6-monooxygenase activity	Cytochrome P450 2C9	Homo sapiens (human)
aromatase activity	Cytochrome P450 2C9	Homo sapiens (human)
heme binding	Cytochrome P450 2C9	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 2C9	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 2B6	Homo sapiens (human)
iron ion binding	Cytochrome P450 2B6	Homo sapiens (human)
testosterone 16-alpha-hydroxylase activity	Cytochrome P450 2B6	Homo sapiens (human)
heme binding	Cytochrome P450 2B6	Homo sapiens (human)
testosterone 16-beta-hydroxylase activity	Cytochrome P450 2B6	Homo sapiens (human)
anandamide 8,9 epoxidase activity	Cytochrome P450 2B6	Homo sapiens (human)
anandamide 11,12 epoxidase activity	Cytochrome P450 2B6	Homo sapiens (human)
anandamide 14,15 epoxidase activity	Cytochrome P450 2B6	Homo sapiens (human)
estrogen 2-hydroxylase activity	Cytochrome P450 2B6	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 2B6	Homo sapiens (human)
arachidonic acid epoxygenase activity	Cytochrome P450 2B6	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 3A5	Homo sapiens (human)
iron ion binding	Cytochrome P450 3A5	Homo sapiens (human)
protein binding	Cytochrome P450 3A5	Homo sapiens (human)
retinoic acid 4-hydroxylase activity	Cytochrome P450 3A5	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 3A5	Homo sapiens (human)
oxygen binding	Cytochrome P450 3A5	Homo sapiens (human)
heme binding	Cytochrome P450 3A5	Homo sapiens (human)
aromatase activity	Cytochrome P450 3A5	Homo sapiens (human)
estrogen 16-alpha-hydroxylase activity	Cytochrome P450 3A5	Homo sapiens (human)
testosterone 6-beta-hydroxylase activity	Cytochrome P450 3A5	Homo sapiens (human)
monooxygenase activity	Cytochrome P450 2C19	Homo sapiens (human)
iron ion binding	Cytochrome P450 2C19	Homo sapiens (human)
steroid hydroxylase activity	Cytochrome P450 2C19	Homo sapiens (human)
oxidoreductase activity	Cytochrome P450 2C19	Homo sapiens (human)
(S)-limonene 6-monooxygenase activity	Cytochrome P450 2C19	Homo sapiens (human)
(S)-limonene 7-monooxygenase activity	Cytochrome P450 2C19	Homo sapiens (human)
oxygen binding	Cytochrome P450 2C19	Homo sapiens (human)
enzyme binding	Cytochrome P450 2C19	Homo sapiens (human)
heme binding	Cytochrome P450 2C19	Homo sapiens (human)
(R)-limonene 6-monooxygenase activity	Cytochrome P450 2C19	Homo sapiens (human)
aromatase activity	Cytochrome P450 2C19	Homo sapiens (human)
long-chain fatty acid omega-1 hydroxylase activity	Cytochrome P450 2C19	Homo sapiens (human)
arachidonic acid epoxygenase activity	Cytochrome P450 2C19	Homo sapiens (human)
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	Cytochrome P450 2C19	Homo sapiens (human)
RNA polymerase II cis-regulatory region sequence-specific DNA binding	Nuclear receptor ROR-gamma	Homo sapiens (human)
DNA-binding transcription factor activity, RNA polymerase II-specific	Nuclear receptor ROR-gamma	Homo sapiens (human)
DNA-binding transcription repressor activity, RNA polymerase II-specific	Nuclear receptor ROR-gamma	Homo sapiens (human)
DNA-binding transcription factor activity	Nuclear receptor ROR-gamma	Homo sapiens (human)
protein binding	Nuclear receptor ROR-gamma	Homo sapiens (human)
oxysterol binding	Nuclear receptor ROR-gamma	Homo sapiens (human)
zinc ion binding	Nuclear receptor ROR-gamma	Homo sapiens (human)
ligand-activated transcription factor activity	Nuclear receptor ROR-gamma	Homo sapiens (human)
sequence-specific double-stranded DNA binding	Nuclear receptor ROR-gamma	Homo sapiens (human)
nuclear receptor activity	Nuclear receptor ROR-gamma	Homo sapiens (human)
solute:inorganic anion antiporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
protein binding	Solute carrier family 22 member 6	Homo sapiens (human)
organic anion transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
prostaglandin transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
alpha-ketoglutarate transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
antiporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
chloride ion binding	Solute carrier family 22 member 6	Homo sapiens (human)
identical protein binding	Solute carrier family 22 member 6	Homo sapiens (human)
xenobiotic transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
sodium-independent organic anion transmembrane transporter activity	Solute carrier family 22 member 6	Homo sapiens (human)
solute:inorganic anion antiporter activity	Solute carrier family 22 member 8	Homo sapiens (human)
organic anion transmembrane transporter activity	Solute carrier family 22 member 8	Homo sapiens (human)
prostaglandin transmembrane transporter activity	Solute carrier family 22 member 8	Homo sapiens (human)
xenobiotic transmembrane transporter activity	Solute carrier family 22 member 8	Homo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Process	via Protein(s)	Taxonomy
endoplasmic reticulum membrane	Cytochrome P450 1A2	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 1A2	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 1A2	Homo sapiens (human)
cytoplasm	Cytochrome P450 3A4	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 3A4	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 3A4	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 2C8	Homo sapiens (human)
plasma membrane	Cytochrome P450 2C8	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C8	Homo sapiens (human)
cytoplasm	Cytochrome P450 2C8	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C8	Homo sapiens (human)
mitochondrion	Cytochrome P450 2D6	Homo sapiens (human)
endoplasmic reticulum	Cytochrome P450 2D6	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 2D6	Homo sapiens (human)
cytoplasm	Cytochrome P450 2D6	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2D6	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 2C9	Homo sapiens (human)
plasma membrane	Cytochrome P450 2C9	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C9	Homo sapiens (human)
cytoplasm	Cytochrome P450 2C9	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C9	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 2B6	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2B6	Homo sapiens (human)
cytoplasm	Cytochrome P450 2B6	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 3A5	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 3A5	Homo sapiens (human)
endoplasmic reticulum membrane	Cytochrome P450 2C19	Homo sapiens (human)
plasma membrane	Cytochrome P450 2C19	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C19	Homo sapiens (human)
intracellular membrane-bounded organelle	Cytochrome P450 2C19	Homo sapiens (human)
cytoplasm	Cytochrome P450 2C19	Homo sapiens (human)
nucleus	Nuclear receptor ROR-gamma	Homo sapiens (human)
nucleoplasm	Nuclear receptor ROR-gamma	Homo sapiens (human)
nuclear body	Nuclear receptor ROR-gamma	Homo sapiens (human)
chromatin	Nuclear receptor ROR-gamma	Homo sapiens (human)
nucleus	Nuclear receptor ROR-gamma	Homo sapiens (human)
plasma membrane	Solute carrier family 22 member 6	Homo sapiens (human)
caveola	Solute carrier family 22 member 6	Homo sapiens (human)
basal plasma membrane	Solute carrier family 22 member 6	Homo sapiens (human)
basolateral plasma membrane	Solute carrier family 22 member 6	Homo sapiens (human)
extracellular exosome	Solute carrier family 22 member 6	Homo sapiens (human)
protein-containing complex	Solute carrier family 22 member 6	Homo sapiens (human)
plasma membrane	Solute carrier family 22 member 8	Homo sapiens (human)
basolateral plasma membrane	Solute carrier family 22 member 8	Homo sapiens (human)
apical plasma membrane	Solute carrier family 22 member 8	Homo sapiens (human)
extracellular exosome	Solute carrier family 22 member 8	Homo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Bioassays (197)

Assay ID	Title	Year	Journal	Article
AID588501	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set	2010	Current protocols in cytometry, Oct, Volume: Chapter 13	Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588501	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set	2006	Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5	Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588501	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set	2010	Assay and drug development technologies, Feb, Volume: 8, Issue:1	High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID588497	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set	2010	Current protocols in cytometry, Oct, Volume: Chapter 13	Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588497	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set	2006	Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5	Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588497	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set	2010	Assay and drug development technologies, Feb, Volume: 8, Issue:1	High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID504810	Antagonists of the Thyroid Stimulating Hormone Receptor: HTS campaign	2010	Endocrinology, Jul, Volume: 151, Issue:7	A small molecule inverse agonist for the human thyroid-stimulating hormone receptor.
AID504812	Inverse Agonists of the Thyroid Stimulating Hormone Receptor: HTS campaign	2010	Endocrinology, Jul, Volume: 151, Issue:7	A small molecule inverse agonist for the human thyroid-stimulating hormone receptor.
AID1745845	Primary qHTS for Inhibitors of ATXN expression
AID588499	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set	2010	Current protocols in cytometry, Oct, Volume: Chapter 13	Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588499	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set	2006	Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5	Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588499	High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set	2010	Assay and drug development technologies, Feb, Volume: 8, Issue:1	High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID651635	Viability Counterscreen for Primary qHTS for Inhibitors of ATXN expression
AID1750639	Direct inhibition of CYP1A2 in human liver microsomes using Phenacetin as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750522	Drug metabolism in mouse hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750585	Half life in Sprague-Dawley rat plasma at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750545	Cmax in CD1 mouse at 30 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750650	Time-dependent inhibition of CYP2B6 in human liver microsomes using bupropion as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750508	Reduction of 2-Methylcitric acid in human hepatocytes derived from methylmalonic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750665	Inhibition of human OATP1B1 assessed as reduction in OATP1B1- mediated E217betaG transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750662	Inhibition of human MATE1 assessed as reduction in MATE1-mediated metformin transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750582	Oral bioavailability in Sprague-Dawley rat at 10 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750515	Reduction of CoASH in human hepatocytes derived from methylmalonic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750611	Ratio of drug level in Gottingen minipig liver to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 48 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750521	Drug metabolism in human hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750609	Ratio of drug level in Gottingen minipig liver to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 8 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750651	Time-dependent inhibition of CYP2C8 in human liver microsomes using amodiaquine as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750556	Volume of distribution of CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750510	Reduction of acetyl-CoA in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750518	Drug metabolism in human hepatocytes derived from propionic acidemia patient assessed as formation of 2,2-dimethylbutanoic acid-CoA pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750583	Oral bioavailability in Sprague-Dawley rat at 50 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750492	Reduction of propionyl-CoA in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750672	Induction of toxicity in minipig assessed as increase in albumin to globin ratio at 300 mg/kg/day	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750606	Drug excretion in Gottingen minipig urine at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750547	Cmax in CD1 mouse at 300 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750551	Tmax in CD1 mouse at 300 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750505	Reduction of 2-Methylcitric acid in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750548	Tmax in CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750624	Drug metabolism in Sprague-Dawley rat urine assessed as HST5040-carnitine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750516	Reduction of CoASH in human hepatocytes derived from methylmalonic acidemia at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750538	Inhibition of human OAT3 assessed as reduction in OAT3-mediated tenofovir transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750617	Stability in rat hepatocytes assessed as unchanged compound at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750649	Time-dependent inhibition of CYP1A2 in human liver microsomes using Phenacetin as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750564	Oral bioavailability in CD1 mouse at 300 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750666	Inhibition of human OATP1B3 assessed as reduction in OATP1B3-mediated E217betaG transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750670	Induction of toxicity in minipig assessed as increase in vacuole size in the epithelial cells of renal tubules at >=50 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750542	Ratio of drug level in erythrocyte to plasma in human	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750494	Reduction of propionyl-CoA in human hepatocytes derived from methylmalonic acidemia pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750607	Drug excretion in Gottingen minipig feces at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750581	Induction of toxicity in minipig assessed as induction of adverse effect at 300 mg/kg/day	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750578	AUC (0 to infinity) in Sprague-Dawley rat at 10 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750576	Clearance in Sprague-Dawley rat at 10 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750656	Time-dependent inhibition of CYP3A5 in human liver microsomes using midazolam as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750642	Direct inhibition of CYP2C9 in human liver microsomes using diclofenac as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750667	Inhibition of human OCT1 assessed as reduction in OCT1-mediated metformin transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750648	Direct inhibition of CYP3A5 in human liver microsomes using testosterone as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750513	Reduction of CoASH in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750634	Drug metabolism in Gottingen minipig plasma assessed as HST5040-glycine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750673	Drug metabolism in minipig assessed as beta-oxidation by measuring phase1 metabolite formation at 10 mg/kg administered single dose using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750496	Reduction of methylmalonyl-CoA in human hepatocytes derived from methylmalonic acidemia at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750594	Tmax in Gottingen minipig at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750633	Drug metabolism in Gottingen minipig plasma assessed as HST5040-carnitine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750612	Ratio of drug level in Gottingen minipig brain to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 48 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750657	Time-dependent inhibition of CYP3A4 in human liver microsomes using testosterone as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750514	Reduction of CoASH in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750641	Direct inhibition of CYP2C8 in human liver microsomes using amodiaquine as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750493	Reduction of propionyl-CoA in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750520	Drug metabolism in human hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750530	Solubility in pH 7-buffered water	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750631	Drug metabolism in Gottingen minipig urine assessed as HST5040-glycine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750636	Drug metabolism in rat hepatocytes assessed as beta-oxidation by measuring phase1 metabolite formation at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750593	Cmax in Yucatan minipig at 300 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750595	Tmax in Yucatan minipig at 20 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750539	Ratio of drug level in erythrocyte to plasma in Sprague-Dawley rat	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750610	Ratio of drug level in Gottingen minipig liver to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 24 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750533	Protein binding in human plasma at 40 uM incubated for 4 hrs by equilibrium dialysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750558	AUC (0 to infinity) in CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750646	Direct inhibition of CYP3A5 in human liver microsomes using midazolam as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750626	Drug metabolism in Sprague-Dawley rat feces assessed as HST5040-glucuronide metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750608	Ratio of drug level in Gottingen minipig liver to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 2 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750549	Tmax in CD1 mouse at 30 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750536	Ratio of drug level in blood to plasma in human	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750570	Tmax in Sprague-Dawley rat at 200 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750618	Stability in minipig hepatocytes assessed as unchanged compound at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750635	Drug metabolism in human hepatocytes assessed as beta-oxidation by measuring phase1 metabolite formation at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750568	Tmax in Sprague-Dawley rat at 10 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750591	Cmax in Yucatan minipig at 50 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750523	Drug metabolism in rat hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750643	Direct inhibition of CYP2C19 in human liver microsomes using S-mephenytoin as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750655	Time-dependent inhibition of CYP3A4 in human liver microsomes using midazolam as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750535	Ratio of drug level in blood to plasma in Gottingen minipig	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750519	Drug metabolism in human hepatocytes derived from methylmalonic acidemia patient assessed as formation of 2,2-dimethylbutanoic acid-CoA pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to contro	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750575	Volume of distribution in Sprague-Dawley rat at 10 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750659	Inhibition of human MDR1 assessed as reduction in NMQ transport at 24000 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750599	AUC (0 to infinity) in Gottingen minipig at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750640	Direct inhibition of CYP2B6 in human liver microsomes using bupropion as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750671	Induction of toxicity in minipig assessed as increase in serum albumin concentration at 300 mg/kg/day	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750628	Drug metabolism in Sprague-Dawley rat feces assessed as HST5040-glycine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750537	Inhibition of human OAT1 assessed as reduction in OAT1-mediated tenofovir transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750664	Inhibition of human OAT3 assessed as reduction in OAT3-mediated tenofovir transport	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750620	Drug metabolism in Sprague-Dawley rat plasma assessed as HST5040-glucuronide metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750512	Reduction of acetyl-CoA in human hepatocytes pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750501	Reduction of acetyl carnitine in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750553	Half life in CD1 mouse at 30 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750527	Dissociation constant, pKa of the compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750569	Tmax in Sprague-Dawley rat at 50 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750638	Drug metabolism in Sprague-Dawley rat assessed as beta-oxidation by measuring phase1 metabolite formation at 10 mg/kg administered single dose using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750668	Inhibition of human OCT2 assessed as reduction in OCT2-mediated metformin transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750579	AUC (0 to infinity) in Sprague-Dawley rat at 50 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750596	Tmax in Yucatan minipig at 50 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750541	Inhibition of human MATE2K assessed as reduction in MATE2K-mediated metformin transport at 14900 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750647	Direct inhibition of CYP3A4 in human liver microsomes using testosterone as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750555	Half life in CD1 mouse at 300 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750540	Ratio of drug level in erythrocyte to plasma in Gottingen minipig	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750625	Drug metabolism in Sprague-Dawley rat urine assessed as HST5040-glycine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750502	Reduction of acetyl carnitine in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750654	Time-dependent inhibition of CYP2D6 in human liver microsomes using bufuralol as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750552	Half life in CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750566	Cmax in Sprague-Dawley rat at 50 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750602	AUC (0 to infinity) in Yucatan minipig at 125 mg/kg, po using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750658	Time-dependent inhibition of CYP3A5 in human liver microsomes using testosterone as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750532	Protein binding in Gottingen minipig plasma at 40 uM incubated for 4 hrs by equilibrium dialysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750563	Oral bioavailability in CD1 mouse at 100 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750621	Drug metabolism in Sprague-Dawley rat plasma assessed as HST5040-carnitine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750504	Reduction of acetyl carnitine in human hepatocytes derived from methylmalonic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750586	Half life in Sprague-Dawley rat blood at 10 mg/kg, po by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750507	Reduction of 2-Methylcitric acid in human hepatocytes derived from methylmalonic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750597	Tmax in Yucatan minipig at 125 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750528	Apparent permeability of the compound across apical to basal side in human Caco-2 cells	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750577	AUC (0 to infinity) in Sprague-Dawley rat at 10 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750491	Reduction of propionyl-CoA in human hepatocytes derived from propionic acidemia patient at 100 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750517	Reduction of CoASH in human hepatocytes at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750644	Direct inhibition of CYP2D6 in human liver microsomes using bufuralol as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750500	Reduction of propionyl carnitine in human hepatocytes derived from methylmalonic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750565	Cmax in Sprague-Dawley rat at 10 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750588	Drug excretion in Sprague-Dawley rat urine at 10 mg/kg, po using radiolabeled compound measured at 1 hr post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750663	Inhibition of human OAT1 assessed as reduction in OAT1-mediated tenofovir transport	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750584	Oral bioavailability in Sprague-Dawley rat at 200 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750511	Reduction of acetyl-CoA in human hepatocytes derived from methylmalonic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750604	Drug level in Gottingen minipig blood at 10 mg/kg, po using radiolabeled compound measured after 96 hrs by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750503	Reduction of acetyl carnitine in human hepatocytes derived from methylmalonic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750669	Induction of toxicity in minipig assessed as increase in number of cells with cytoplasmic vacuoles in renal tubules at >=50 mg/kg/day	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750499	Reduction of propionyl carnitine in human hepatocytes derived from methylmalonic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750554	Half life in CD1 mouse at 100 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750660	Inhibition of human BCRP assessed as reduction in E3S transport at 24000 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750590	Cmax in Yucatan minipig at 20 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750587	Ratio of drug level in liver to plasma in Sprague-Dawley rat at 10 mg/kg, po measured at 1 hr post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750574	Half life in Sprague-Dawley rat at 10 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750529	Apparent permeability across apical to basal side in human MDCK2-MDR1	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750526	Drug metabolism in mini-pig hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750661	Inhibition of human BSEP assessed as reduction in BSEP-mediated taurocholate transport at 345 uM relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750561	AUC (0 to infinity) in CD1 mouse at 300 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750534	Ratio of drug level in blood to plasma in Sprague-Dawley rat	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750497	Reduction of propionyl carnitine in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750557	Clearance in CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750573	Half life in Sprague-Dawley rat at 200 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750572	Half life in Sprague-Dawley rat at 50 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750600	AUC (0 to infinity) in Yucatan minipig at 20 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750495	Reduction of methylmalonyl-CoA in human hepatocytes derived from methylmalonic acidemia pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750567	Cmax in Sprague-Dawley rat at 200 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750615	Cytotoxicity against rat hepatocytes assessed as reduction in cell viability using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750598	Tmax in Yucatan minipig at 300 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID167125	Eye irritation potential accessed using Draize in vivo rabbit eye irritation test	2003	Journal of medicinal chemistry, Apr-10, Volume: 46, Issue:8	Mapping property distributions of molecular surfaces: algorithm and evaluation of a novel 3D quantitative structure-activity relationship technique.
AID1750622	Drug metabolism in Sprague-Dawley rat plasma assessed as HST5040-glycine metabolite formation at 10 mg/kg administered as single dose using radiolabeled compound by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750652	Time-dependent inhibition of CYP2C9 in human liver microsomes using diclofenac as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750524	Drug metabolism in Beagle dog hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750543	Reduction of propionyl-CoA in human hepatocytes derived from methylmalonic acidemia at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750525	Drug metabolism in cynomolgus monkey hepatocytes assessed as formation of 2,2-dimethylbutanoic acid-CoA incubated for 1 hrs by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750614	Cytotoxicity against human hepatocytes assessed as reduction in cell viability using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750544	Cmax in CD1 mouse at 30 mg/kg, iv	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750619	Stability in human hepatocytes assessed as unchanged compound at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750562	Oral bioavailability in CD1 mouse at 30 mg/kg	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750580	AUC (0 to infinity) in Sprague-Dawley rat at 200 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750630	Drug metabolism in Gottingen minipig urine assessed as HST5040-carnitine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750613	Ratio of drug level in Gottingen minipig spinal cord to plasma ratio at 10 mg/kg, po using radiolabeled compound measured at 48 hrs post dose	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750629	Drug metabolism in Gottingen minipig urine assessed as HST5040-glucuronide metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750571	Half life in Sprague-Dawley rat at 10 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750509	Reduction of acetyl-CoA in human hepatocytes derived from propionic acidemia patient pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750601	AUC (0 to infinity) in Yucatan minipig at 50 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750498	Reduction of propionyl carnitine in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750627	Drug metabolism in Sprague-Dawley rat feces assessed as HST5040-carnitine metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750531	Protein binding in Sprague-Dawley rat plasma at 40 uM incubated for 4 hrs by equilibrium dialysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750623	Drug metabolism in Sprague-Dawley rat urine assessed as HST5040-glucuronide metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750589	Cmax in Gottingen minipig at 10 mg/kg, po using radiolabeled compound by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750592	Cmax in Yucatan minipig at 125 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750605	Drug level in Gottingen minipig blood at 10 mg/kg, po using radiolabeled compound measured after 72 hrs by quantitative whole-body autoradiography	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750506	Reduction of 2-Methylcitric acid in human hepatocytes derived from propionic acidemia patient at 30 uM pretreated for 30 mins followed by 13C-isoleucine addition and measured after 1 hr by MS/MS analysis relative to control	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750559	AUC (0 to infinity) in CD1 mouse at 30 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750637	Drug metabolism in minipig hepatocytes assessed as beta-oxidation by measuring phase1 metabolite formation at 50 uM measured after 4 hrs	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750546	Cmax in CD1 mouse at 100 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750560	AUC (0 to infinity) in CD1 mouse at 100 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750550	Tmax in CD1 mouse at 100 mg/kg, po	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750603	AUC (0 to infinity) in Yucatan minipig at 300 mg/kg, po using radiolabeled compound administered for 3 weeks	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750645	Direct inhibition of CYP3A4 in human liver microsomes using midazolam as substrate	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750653	Time-dependent inhibition of CYP2C19 in human liver microsomes using S-mephenytoin as substrate preincubated for 30 mins	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750632	Drug metabolism in Gottingen minipig plasma assessed as HST5040-glucuronide metabolite formation at 10 mg/kg administered as single dose by LC/MS analysis	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
AID1750616	Cytotoxicity against minipig hepatocytes assessed as reduction in cell viability using radiolabeled compound	2021	Journal of medicinal chemistry, 04-22, Volume: 64, Issue:8	Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
[information is prepared from bioassay data collected from National Library of Medicine (NLM), extracted Dec-2023]

Timeframe	Studies, This Drug (%)	All Drugs %
pre-1990	0 (0.00)	18.7374
1990's	0 (0.00)	18.2507
2000's	4 (18.18)	29.6817
2010's	14 (63.64)	24.3611
2020's	4 (18.18)	2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Publication Type	This drug (%)	All Drugs (%)
Trials	7 (30.43%)	5.53%
Reviews	1 (4.35%)	6.00%
Case Studies	0 (0.00%)	4.05%
Observational	0 (0.00%)	0.25%
Other	15 (65.22%)	84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Substance	Relationship Strength	Studies	Trials	Classes	Roles
3-phenylpropionic acid 3-phenylpropionic acid: RN given refers to parent cpd. 3-phenylpropionic acid : A monocarboxylic acid that is propionic acid substituted at position 3 by a phenyl group.	2.31	1	0	benzenes; monocarboxylic acid	antifungal agent; human metabolite; plant metabolite
acetone methyl ketone : A ketone of formula RC(=O)CH3 (R =/= H).	2.01	1	0	ketone body; methyl ketone; propanones; volatile organic compound	EC 3.5.1.4 (amidase) inhibitor; human metabolite; polar aprotic solvent
1-butanol 1-Butanol: A four carbon linear hydrocarbon that has a hydroxy group at position 1.. butan-1-ol : A primary alcohol that is butane in which a hydrogen of one of the methyl groups is substituted by a hydroxy group. It it produced in small amounts in humans by the gut microbes.	2.01	1	0	alkyl alcohol; primary alcohol; short-chain primary fatty alcohol	human metabolite; mouse metabolite; protic solvent
ethyl 2-methyl-3-oxobutanoate [no description available]	2.01	1	0
glycerol Moon: The natural satellite of the planet Earth. It includes the lunar cycles or phases, the lunar month, lunar landscapes, geography, and soil.	2.01	1	0	alditol; triol	algal metabolite; detergent; Escherichia coli metabolite; geroprotector; human metabolite; mouse metabolite; osmolyte; Saccharomyces cerevisiae metabolite; solvent
phenylacetic acid phenylacetic acid : A monocarboxylic acid that is toluene in which one of the hydrogens of the methyl group has been replaced by a carboxy group.	2.31	1	0	benzenes; monocarboxylic acid; phenylacetic acids	allergen; Aspergillus metabolite; auxin; EC 6.4.1.1 (pyruvate carboxylase) inhibitor; Escherichia coli metabolite; human metabolite; plant growth retardant; plant metabolite; Saccharomyces cerevisiae metabolite; toxin
propylene glycol Propylene Glycol: A clear, colorless, viscous organic solvent and diluent used in pharmaceutical preparations.. propane-1,2-diol : The simplest member of the class of propane-1,2-diols, consisting of propane in which a hydrogen at position 1 and a hydrogen at position 2 are substituted by hydroxy groups. A colourless, viscous, hygroscopic, low-melting (-59degreeC) and high-boiling (188degreeC) liquid with low toxicity, it is used as a solvent, emulsifying agent, and antifreeze.	2.01	1	0	glycol; propane-1,2-diols	allergen; human xenobiotic metabolite; mouse metabolite; protic solvent
toluene methylbenzene : Any alkylbenzene that is benzene substituted with one or more methyl groups.	2.01	1	0	methylbenzene; toluenes; volatile organic compound	cholinergic antagonist; fuel additive; neurotoxin; non-polar solvent
benserazide Benserazide: An inhibitor of DOPA DECARBOXYLASE that does not enter the central nervous system. It is often given with LEVODOPA in the treatment of parkinsonism to prevent the conversion of levodopa to dopamine in the periphery, thereby increasing the amount that reaches the central nervous system and reducing the required dose. It has no antiparkinson actions when given alone.. benserazide : A carbohydrazide that results from the formal condensation of the carboxy group of DL-serine with the primary amino group of 4-(hydrazinylmethyl)benzene-1,2,3-triol. An aromatic-L-amino-acid decarboxylase inhibitor (DOPA decarboxylase inhibitor) that does not enter the central nervous system, it is used as its hydrochloride salt as an adjunct to levodopa in the treatment of parkinsonism. By preventing the conversion of levodopa to dopamine in the periphery, it causes an increase in the amount of levodopa reaching the central nervous system and so reduces the required dose. Benserazide has no antiparkinson actions when given alone.	3.04	1	0	carbohydrazide; catechols; primary alcohol; primary amino compound	antiparkinson drug; dopaminergic agent; EC 4.1.1.28 (aromatic-L-amino-acid decarboxylase) inhibitor
verapamil Verapamil: A calcium channel blocker that is a class IV anti-arrhythmia agent.. verapamil : A racemate comprising equimolar amounts of dexverapamil and (S)-verapamil. An L-type calcium channel blocker of the phenylalkylamine class, it is used (particularly as the hydrochloride salt) in the treatment of hypertension, angina pectoris and cardiac arrhythmia, and as a preventive medication for migraine.. 2-(3,4-dimethoxyphenyl)-5-{[2-(3,4-dimethoxyphenyl)ethyl](methyl)amino}-2-(propan-2-yl)pentanenitrile : A tertiary amino compound that is 3,4-dimethoxyphenylethylamine in which the hydrogens attached to the nitrogen are replaced by a methyl group and a 4-cyano-4-(3,4-dimethoxyphenyl)-5-methylhexyl group.	2.01	1	0	aromatic ether; nitrile; polyether; tertiary amino compound
valproic acid Valproic Acid: A fatty acid with anticonvulsant and anti-manic properties that is used in the treatment of EPILEPSY and BIPOLAR DISORDER. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GAMMA-AMINOBUTYRIC ACID levels in the brain or by altering the properties of VOLTAGE-GATED SODIUM CHANNELS.. valproic acid : A branched-chain saturated fatty acid that comprises of a propyl substituent on a pentanoic acid stem.	2.31	1	0	branched-chain fatty acid; branched-chain saturated fatty acid	anticonvulsant; antimanic drug; EC 3.5.1.98 (histone deacetylase) inhibitor; GABA agent; neuroprotective agent; psychotropic drug; teratogenic agent
hydroxyurea [no description available]	7.26	3	2	one-carbon compound; ureas	antimetabolite; antimitotic; antineoplastic agent; DNA synthesis inhibitor; EC 1.17.4.1 (ribonucleoside-diphosphate reductase) inhibitor; genotoxin; immunomodulator; radical scavenger; teratogenic agent
2-propanol 2-Propanol: An isomer of 1-PROPANOL. It is a colorless liquid having disinfectant properties. It is used in the manufacture of acetone and its derivatives and as a solvent. Topically, it is used as an antiseptic.. propan-2-ol : A secondary alcohol that is propane in which one of the hydrogens attached to the central carbon is substituted by a hydroxy group.	2.01	1	0	secondary alcohol; secondary fatty alcohol	protic solvent
methoctramine [no description available]	2.01	1	0	aromatic ether; tetramine	muscarinic antagonist
entinostat [no description available]	2.13	1	0	benzamides; carbamate ester; primary amino compound; pyridines; substituted aniline	antineoplastic agent; apoptosis inducer; EC 3.5.1.98 (histone deacetylase) inhibitor
4-phenylbutyric acid 4-phenylbutyric acid: RN refers to the parent cpd. 4-phenylbutyric acid : A monocarboxylic acid the structure of which is that of butyric acid substituted with a phenyl group at C-4. It is a histone deacetylase inhibitor that displays anticancer activity. It inhibits cell proliferation, invasion and migration and induces apoptosis in glioma cells. It also inhibits protein isoprenylation, depletes plasma glutamine, increases production of foetal haemoglobin through transcriptional activation of the gamma-globin gene and affects hPPARgamma activation.	2.31	1	0	monocarboxylic acid	antineoplastic agent; apoptosis inducer; EC 3.5.1.98 (histone deacetylase) inhibitor; prodrug
hydrocortisone acetate hydrocortisone acetate: RN given refers to cpd without isomeric designation	2.01	1	0	cortisol ester; tertiary alpha-hydroxy ketone
corticosterone [no description available]	2.01	1	0	11beta-hydroxy steroid; 20-oxo steroid; 21-hydroxy steroid; 3-oxo-Delta(4) steroid; C21-steroid; glucocorticoid; primary alpha-hydroxy ketone	human metabolite; mouse metabolite
prednisolone Prednisolone: A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.. prednisolone : A glucocorticoid that is prednisone in which the oxo group at position 11 has been reduced to the corresponding beta-hydroxy group. It is a drug metabolite of prednisone.	2.01	1	0	11beta-hydroxy steroid; 17alpha-hydroxy steroid; 20-oxo steroid; 21-hydroxy steroid; 3-oxo-Delta(1),Delta(4)-steroid; C21-steroid; glucocorticoid; primary alpha-hydroxy ketone; tertiary alpha-hydroxy ketone	adrenergic agent; anti-inflammatory drug; antineoplastic agent; drug metabolite; environmental contaminant; immunosuppressive agent; xenobiotic
estriol hormonin: estrogen replacement; each tablet contains 600 ug micronized 17beta-estradiol, 270 ug estriol and 1.4 mg estrone. chlorapatite : A phosphate mineral with the formula Ca5(PO4)3Cl.	2.01	1	0	16alpha-hydroxy steroid; 17beta-hydroxy steroid; 3-hydroxy steroid	estrogen; human metabolite; human xenobiotic metabolite; mouse metabolite
aldosterone [no description available]	2.01	1	0	11beta-hydroxy steroid; 18-oxo steroid; 20-oxo steroid; 21-hydroxy steroid; 3-oxo-Delta(4) steroid; C21-steroid hormone; mineralocorticoid; primary alpha-hydroxy ketone; steroid aldehyde	human metabolite; mouse metabolite
estrone Hydroxyestrones: Estrone derivatives substituted with one or more hydroxyl groups in any position. They are important metabolites of estrone and other estrogens.	2.01	1	0	17-oxo steroid; 3-hydroxy steroid; phenolic steroid; phenols	antineoplastic agent; bone density conservation agent; estrogen; human metabolite; mouse metabolite
androsterone [no description available]	2.01	1	0	17-oxo steroid; 3alpha-hydroxy steroid; androstanoid; C19-steroid	androgen; anticonvulsant; human blood serum metabolite; human metabolite; human urinary metabolite; mouse metabolite; pheromone
etiocholanolone Etiocholanolone: The 5-beta-reduced isomer of ANDROSTERONE. Etiocholanolone is a major metabolite of TESTOSTERONE and ANDROSTENEDIONE in many mammalian species including humans. It is excreted in the URINE.. 3alpha-hydroxy-5beta-androstan-17-one : An androstanoid that is 5beta-androstane substituted by an alpha-hydroxy group at position 3 and an oxo group at position 17. It is a metabolite of testosterone in mammals.	2.01	1	0	17-oxo steroid; 3alpha-hydroxy steroid; androstanoid	human metabolite; mouse metabolite
dehydroepiandrosterone Dehydroepiandrosterone: A major C19 steroid produced by the ADRENAL CORTEX. It is also produced in small quantities in the TESTIS and the OVARY. Dehydroepiandrosterone (DHEA) can be converted to TESTOSTERONE; ANDROSTENEDIONE; ESTRADIOL; and ESTRONE. Most of DHEA is sulfated (DEHYDROEPIANDROSTERONE SULFATE) before secretion.. dehydroepiandrosterone : An androstanoid that is androst-5-ene substituted by a beta-hydroxy group at position 3 and an oxo group at position 17. It is a naturally occurring steroid hormone produced by the adrenal glands.	2.01	1	0	17-oxo steroid; 3beta-hydroxy-Delta(5)-steroid; androstanoid	androgen; human metabolite; mouse metabolite
androstenedione Androstenedione: A delta-4 C19 steroid that is produced not only in the TESTIS, but also in the OVARY and the ADRENAL CORTEX. Depending on the tissue type, androstenedione can serve as a precursor to TESTOSTERONE as well as ESTRONE and ESTRADIOL.. androst-4-ene-3,17-dione : A 3-oxo Delta(4)-steroid that is androst-4-ene substituted by oxo groups at positions 3 and 17. It is a steroid hormone synthesized in the adrenal glands and gonads.	2.01	1	0	17-oxo steroid; 3-oxo-Delta(4) steroid; androstanoid	androgen; Daphnia magna metabolite; human metabolite; mouse metabolite
desoxycorticosterone Desoxycorticosterone: A steroid metabolite that is the 11-deoxy derivative of CORTICOSTERONE and the 21-hydroxy derivative of PROGESTERONE	2.01	1	0	20-oxo steroid; 21-hydroxy steroid; 3-oxo-Delta(4) steroid; mineralocorticoid; primary alpha-hydroxy ketone	human metabolite; mouse metabolite
17-alpha-hydroxyprogesterone 17alpha-hydroxyprogesterone : A 17alpha-hydroxy steroid that is the 17alpha-hydroxy derivative of progesterone.	2.01	1	0	17alpha-hydroxy-C21-steroid; 17alpha-hydroxy steroid; tertiary alpha-hydroxy ketone	human metabolite; metabolite; mouse metabolite; progestin
pivalic acid pivalic acid: RN given refers to parent cpd; structure. pivalic acid : A branched, short-chain fatty acid composed of propanoic acid having two methyl substituents at the 2-position.	2.31	1	0	branched-chain saturated fatty acid; methyl-branched fatty acid; short-chain fatty acid
isobutyl alcohol isobutyl alcohol: RN given refers to parent cpd	2.01	1	0	alkyl alcohol; primary alcohol	Saccharomyces cerevisiae metabolite
methylethyl ketone methylethyl ketone: solvent; colorless synthetic resins, smokeless powders; may be irritating to eyes, mucous membranes; may be toxic in high concentrations; structure. butanone : Any ketone that is butane substituted by an oxo group at unspecified position.. butan-2-one : A dialkyl ketone that is a four-carbon ketone carrying a single keto- group at position C-2.	2.01	1	0	butanone; dialkyl ketone; methyl ketone; volatile organic compound	bacterial metabolite; polar aprotic solvent
methyl acetate methyl acetate : An acetate ester resulting from the formal condensation of acetic acid with methanol. A low-boiling (57 degreeC) colourless, flammable liquid, it is used as a solvent for many resins and oils.	2.01	1	0	acetate ester; methyl ester; volatile organic compound	EC 3.4.19.3 (pyroglutamyl-peptidase I) inhibitor; fragrance; polar aprotic solvent
methylcyclopentane methylcyclopentane: toxic; RN 96-37-7. methylcyclopentane : A cycloalkane that is cyclopentane substituted by a single methyl group.	2.01	1	0	cycloalkane; volatile organic compound	human metabolite; plant metabolite
2-methylvaleric acid 2-methylvaleric acid : A methyl-branched fatty acid that is pentanoic acid which carries a methyl group at position 2.	2.31	1	0	branched-chain saturated fatty acid; methyl-branched fatty acid; monocarboxylic acid; short-chain fatty acid	flavouring agent; fragrance; plant metabolite
styrene Styrene: A colorless, toxic liquid with a strong aromatic odor. It is used to make rubbers, polymers and copolymers, and polystyrene plastics.. styrene : A vinylarene that is benzene carrying a vinyl group. It has been isolated from the benzoin resin produced by Styrax species.	2.01	1	0	styrenes; vinylarene; volatile organic compound	mouse metabolite; mutagen; plant metabolite
2-ethylhexanol [no description available]	2.01	1	0	primary alcohol	plant metabolite; volatile oil component
4-xylene p-xylene : A xylene with methyl groups at positions 1 and 4.	2.01	1	0	xylene
2-methylpentane Hexanes: Six-carbon saturated hydrocarbon group of the methane series. Include isomers and derivatives. Various polyneuropathies are caused by hexane poisoning.	2.01	1	0	alkane
methyl isobutyl ketone [no description available]	2.01	1	0	ketone
cyclohexanol Cyclohexanols: Monohydroxy derivatives of cyclohexanes that contain the general formula R-C6H11O. They have a camphorlike odor and are used in making soaps, insecticides, germicides, dry cleaning, and plasticizers.. cyclohexanols : An alcohol in which one or more hydroxy groups are attached to a cyclohexane skeleton.	2.01	1	0	cyclohexanols; secondary alcohol	solvent
2-heptanone heptan-2-one : A dialkyl ketone with methyl and pentyl as the alkyl groups.	2.01	1	0	dialkyl ketone; methyl ketone	mouse metabolite; pheromone
1-hexanol 1-hexanol: RN given refers to parent cpd. hexanol : A fatty alcohol consisting of a hydroxy function at any position of an unbranched saturated chain of six carbon atoms.. hexan-1-ol : A primary alcohol that is hexane substituted by a hydroxy group at position 1.	2.01	1	0	hexanol; primary alcohol	alarm pheromone; antibacterial agent; fragrance; plant metabolite
n-butoxyethanol n-butoxyethanol: RN given refers to parent cpd; structure. 2-butoxyethanol : A primary alcohol that is ethanol in which one of the methyl hydrogens is replaced by a butoxy group. A high-boiling (171degreeC) colourless liquid, it is used as a solvent for paints and inks, as well as in some dry cleaning solutions.	2.01	1	0	glycol ether; primary alcohol	protic solvent
n-dodecane dodecane : A straight-chain alkane with 12 carbon atoms. It has been isolated from the essential oils of various plants including Zingiber officinale (ginger).	2.01	1	0	alkane	plant metabolite
2-methylbutanoic acid 2-methylbutanoic acid: RN given refers to parent cpd without isomeric designation. 2-methylbutyric acid : A methylbutyric acid comprising a butyric acid core carrying a 2-methyl substituent. Produced from amino acid leucine during nutrient starvation in bacteria.	2.31	1	0	methylbutyric acid	bacterial metabolite; human metabolite
sec-butylbenzene sec-butylbenzene : An alkylbenzene that is benzene substituted by a butan-2-yl group.	2.01	1	0	alkylbenzene
ethyl acetate ethyl acetate : The acetate ester formed between acetic acid and ethanol.	2.01	1	0	acetate ester; ethyl ester; volatile organic compound	EC 3.4.19.3 (pyroglutamyl-peptidase I) inhibitor; metabolite; polar aprotic solvent; Saccharomyces cerevisiae metabolite
pregnenolone [no description available]	2.01	1	0	20-oxo steroid; 3beta-hydroxy-Delta(5)-steroid; C21-steroid	human metabolite; mouse metabolite
4-fluoroaniline 4-fluoroaniline: chemical intermediate manufactured by the Halex process; RN given refers to parent cpd; structure given in first source. 4-fluoroaniline : A primary arylamine that is the derivative of aniline in which the hydrogen at position 4 has been substituted by fluorine. It is used as an intermediate in the manufacture of pharmaceuticals, herbicides and plant growth regulators.	2.01	1	0	fluoroaniline; primary arylamine
nandrolone Nandrolone: C18 steroid with androgenic and anabolic properties. It is generally prepared from alkyl ethers of ESTRADIOL to resemble TESTOSTERONE but less one carbon at the 19 position.. nandrolone : A 3-oxo Delta(4)-steroid that is estr-4-en-3-one substituted by a beta-hydroxy group at position 17.	2.01	1	0	17beta-hydroxy steroid; 3-oxo-Delta(4) steroid; anabolic androgenic steroid	human metabolite
alpha-aminopyridine alpha-aminopyridine: RN given refers to parent cpd; structure in Merck Index, 9th ed, #485. aminopyridine : Compounds containing a pyridine skeleton substituted by one or more amine groups.	2.13	1	0
androstenediol Androstenediol: An intermediate in TESTOSTERONE biosynthesis, found in the TESTIS or the ADRENAL GLANDS. Androstenediol, derived from DEHYDROEPIANDROSTERONE by the reduction of the 17-keto group (17-HYDROXYSTEROID DEHYDROGENASES), is converted to TESTOSTERONE by the oxidation of the 3-beta hydroxyl group to a 3-keto group (3-HYDROXYSTEROID DEHYDROGENASES).. androst-5-ene-3beta,17beta-diol : A 3beta-hydroxy-Delta(5)-steroid that is 3beta-hydroxyandrost-5-ene carrying an additional hydroxy group at position 17beta.	2.01	1	0	17beta-hydroxy steroid; 3beta-hydroxy-Delta(5)-steroid	androgen; human metabolite; mouse metabolite; radiation protective agent
dihydrotestosterone Dihydrotestosterone: A potent androgenic metabolite of TESTOSTERONE. It is produced by the action of the enzyme 3-OXO-5-ALPHA-STEROID 4-DEHYDROGENASE.. 17beta-hydroxyandrostan-3-one : A 17beta-hydroxy steroid that is testosterone in which the 4-5 double bond has been reduced to a single bond with unspecified configuration at position 5.. 17beta-hydroxy-5alpha-androstan-3-one : A 17beta-hydroxy steroid that is testosterone in which the 4,5 double bond has been reduced to a single bond with alpha-configuration at position 5.	2.01	1	0	17beta-hydroxy steroid; 17beta-hydroxyandrostan-3-one; 3-oxo-5alpha-steroid	androgen; Daphnia magna metabolite; human metabolite; mouse metabolite
3-methylhexane 3-methylhexane: a biliary biomarker for cholangiocarcinoma. 3-methylhexane : An alkane that is hexane substituted by a methyl group at position 3.	2.01	1	0	alkane; volatile organic compound	human metabolite
algestone Algestone: A synthetic progestational dihydroxy derivative of PROGESTERONE. Its acetonide possesses anti-inflammatory properties.. algestone : A C21-steroid that is pregn-4-ene substituted by oxo groups at position 3 and 20 and hydroxy groups at positions 16 and 17.	2.01	1	0	16alpha-hydroxy steroid; 17-hydroxy steroid; 20-oxo steroid; 3-oxo-Delta(4) steroid; C21-steroid; tertiary alpha-hydroxy ketone	progestin
3-ethyltoluene [no description available]	2.01	1	0	toluenes
ethoxyacetic acid [no description available]	2.01	1	0	carboxylic acid
cyclopropanecarboxylic acid cyclopropanecarboxylic acid: 0	2.31	1	0	cyclopropanes; monocarboxylic acid
2-tert-butylhydroquinone 2-tert-butylhydroquinone: an anticarcinogenic and chemopreventive agent. 2-tert-butylhydroquinone : A member of the class of hydroquinones in which one of the ring hydrogens of hydroquinone is replaced by a tert-butyl group.	2.13	1	0	hydroquinones	food antioxidant
butyl acetate butyl acetate: structure. butyl acetate : The acetate ester of butanol.	2.01	1	0	acetate ester	metabolite
simvastatin Simvastatin: A derivative of LOVASTATIN and potent competitive inhibitor of 3-hydroxy-3-methylglutaryl coenzyme A reductase (HYDROXYMETHYLGLUTARYL COA REDUCTASES), which is the rate-limiting enzyme in cholesterol biosynthesis. It may also interfere with steroid hormone production. Due to the induction of hepatic LDL RECEPTORS, it increases breakdown of LDL CHOLESTEROL.. simvastatin : A member of the class of hexahydronaphthalenes that is lovastatin in which the 2-methylbutyrate ester moiety has been replaced by a 2,2-dimethylbutyrate ester group. It is used as a cholesterol-lowering and anti-cardiovascular disease drug.	2.13	1	0	delta-lactone; fatty acid ester; hexahydronaphthalenes; statin (semi-synthetic)	EC 1.1.1.34/EC 1.1.1.88 (hydroxymethylglutaryl-CoA reductase) inhibitor; EC 3.4.24.83 (anthrax lethal factor endopeptidase) inhibitor; ferroptosis inducer; geroprotector; prodrug
1-nitro-2,4-difluorobenzene 1-nitro-2,4-difluorobenzene: standard antigen; structure	2.01	1	0
propionyl-coenzyme a propionyl-coenzyme A: RN given refers to parent cpd	2.31	1	0	acyl-CoA	Escherichia coli metabolite; metabolite; mouse metabolite
11-ketoprogesterone 11-ketoprogesterone: structure	2.01	1	0	corticosteroid hormone
2 alpha-methyl-9 alpha-fluorocortisol [no description available]	2.01	1	0
3 alpha,17 alpha-dihydroxy-5 beta-pregnan-20-one 3alpha,17alpha-dihydroxy-5beta-pregnan-20-one : A 17alpha-hydroxy steroid that is 3alpha-hydroxy-5beta-pregnan-20-one carrying an additional hydroxy group at position 17alpha.	2.01	1	0	17alpha-hydroxy steroid; 20-oxo steroid; 3alpha-hydroxy steroid; C21-steroid; corticosteroid hormone	human urinary metabolite
w 84 [no description available]	2.01	1	0
heptane-1,7-bis(dimethyl-3'-phthalimidopropylammonium) heptane-1,7-bis(dimethyl-3'-phthalimidopropylammonium): RN given refers to parent cpd	2.01	1	0
cortisone [no description available]	2.01	1	0	11-oxo steroid; 17alpha-hydroxy steroid; 20-oxo steroid; 21-hydroxy steroid; 3-oxo-Delta(4) steroid; C21-steroid; glucocorticoid; primary alpha-hydroxy ketone; tertiary alpha-hydroxy ketone	human metabolite; mouse metabolite
19-norprogesterone 19-norprogesterone: RN given refers to cpd without isomeric designation	2.01	1	0	corticosteroid hormone
5 alpha-androstane-3 beta,17 beta-diol 5alpha-androstane-3beta,17beta-diol : An androstane-3,17-diol that is 5alpha-androstane substituted by beta-hydroxy groups at positions 3 and 17. It is a metabolite of dihydrotestosterone.	2.01	1	0	17beta-hydroxy steroid; 3beta-hydroxy steroid; androstane-3,17-diol	Daphnia magna metabolite; human metabolite
cortodoxone Cortodoxone: 17,21-Dihydroxypregn-4-ene-3,20-dione. A 17-hydroxycorticosteroid with glucocorticoid and anti-inflammatory activities.. 11-deoxycortisol : A deoxycortisol that is cortisol in which the hydroxy group at position 11 has been replaced by a hydrogen.	2.01	1	0	deoxycortisol; glucocorticoid; primary alpha-hydroxy ketone; tertiary alpha-hydroxy ketone	human metabolite; mouse metabolite
resveratrol trans-resveratrol : A resveratrol in which the double bond has E configuration.	3.7	1	0	resveratrol	antioxidant; phytoalexin; plant metabolite; quorum sensing inhibitor; radical scavenger
cadinol cadinol: from callus cultures of Chamomilla recutita (Asteraceae)	2.01	1	0
16-methylprogesterone, (16alpha)-isomer [no description available]	2.01	1	0
methylmalonyl-coenzyme a [no description available]	2.31	1	0

Condition	Relationship Strength	Studies	Trials
Innate Inflammatory Response [description not available]	2.05	1	0
Inflammation A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.	2.05	1	0
Acidemia Propionic [description not available]	2.31	1	0
Amino Acid Metabolism Disorders, Inborn [description not available]	2.31	1	0
Propionic Acidemia Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.	2.31	1	0
Anemia, Cooley's [description not available]	9.56	8	7
beta-Thalassemia A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent.	9.56	8	7
Diabetes Mellitus, Adult-Onset [description not available]	2.6	1	0
Diabetes Mellitus, Type 2 A subclass of DIABETES MELLITUS that is not INSULIN-responsive or dependent (NIDDM). It is characterized initially by INSULIN RESISTANCE and HYPERINSULINEMIA; and eventually by GLUCOSE INTOLERANCE; HYPERGLYCEMIA; and overt diabetes. Type II diabetes mellitus is no longer considered a disease exclusively found in adults. Patients seldom develop KETOSIS but often exhibit OBESITY.	2.6	1	0
Obesity A status with BODY WEIGHT that is grossly above the recommended standards, usually due to accumulation of excess FATS in the body. The standards may vary with age, sex, genetic or cultural background. In the BODY MASS INDEX, a BMI greater than 30.0 kg/m2 is considered obese, and a BMI greater than 40.0 kg/m2 is considered morbidly obese (MORBID OBESITY).	2.6	1	0
HbS Disease [description not available]	8.72	7	6
Sickle Cell Trait The condition of being heterozygous for hemoglobin S.	7.51	4	4
Anemia, Sickle Cell A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.	8.72	7	6
Gastritis Inflammation of the GASTRIC MUCOSA, a lesion observed in a number of unrelated disorders.	7.51	4	4
Hemoglobinopathies A group of inherited disorders characterized by structural alterations within the hemoglobin molecule.	3.45	2	0
Body Weight The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms.	2.06	1	0
Sensitivity and Specificity Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)	2.04	1	0

2,2-dimethylbutyric acid

Description

Cross-References

Synonyms (59)

Research Excerpts

Toxicity

Pharmacokinetics

Bioavailability

Dosage Studied

Roles (1)

Drug Classes (1)

Protein Targets (16)

Potency Measurements

Inhibition Measurements

Biological Processes (70)

Molecular Functions (55)

Ceullar Components (16)

Bioassays (197)

Research

Studies (22)

Market Indicators

Research Demand Index: 22.93

Study Types

2,2-dimethylbutyric acid

Description

Cross-References

Synonyms (59)

Research Excerpts

Toxicity

Pharmacokinetics

Bioavailability

Dosage Studied

Roles (1)

Drug Classes (1)

Protein Targets (16)

Potency Measurements

Inhibition Measurements

Biological Processes (70)

Molecular Functions (55)

Ceullar Components (16)

Bioassays (197)

Research

Studies (22)

Market Indicators

Research Demand Index: 22.93

Study Types

Related Drugs (76)

Related Conditions (17)