Inborn Urea Cycle Disorder

Synonym
Urea Cycle Disorders
Inborn Urea Cycle Disorder

Excerpt	Reference
"Urea cycle disorders are inborn errors of metabolism that, in rare cases, can present for the first time in adulthood."	( Blair, NF; Cremer, PD; Tchan, MC, 2015)
"The urea cycle disorders are a group of inherited biochemical diseases caused by a complete or partial deficiency of any one of the enzymes or transport proteins required to convert toxic ammonia into urea and to produce arginine and citrulline."	( Mew, NA; Summar, ML, 2018)
"Urea cycle disorders are congenital metabolism errors that affect ammonia elimination."	( Del Río, C; Martín-Hernández, E; Quijada-Fraile, P; Rubio, P; Ruiz, A, 2020)

Excerpt	Reference
"Available medications for treating urea cycle disorders include sodium benzoate (BA), sodium phenylacetate (PAA), and sodium phenylbutyrate (PBA) and are given to provide alternate routes for disposition of waste nitrogen excretion."	( Herebian, D; Laryea, MD; Mayatepek, E; Meissner, T, 2010)
"Both are approved for treatment of urea cycle disorders (UCDs) - rare genetic disorders characterized by hyperammonemia."	( Bartley, J; Berquist, W; Berry, SA; Brown, RS; Coakley, D; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Ghabril, M; Harding, C; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mantry, P; McCandless, SE; Milikien, DA; Mokhtarani, M; Moors, T; Nagamani, SC; Norris, C; Rhead, W; Rockey, DC; Scharschmidt, BF; Schulze, A; Smith, W; Vierling, JM, 2013)
"4-PBA is currently used clinically to treat urea cycle disorders under the trade name Buphenyl."	( Ask, K; Ayaub, EA; Dickhout, JG; Kolb, PS; Yum, V; Zhou, W, 2015)

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	0 (0.00)	23.3326
1990's	0 (0.00)	12.5806
2000's	1 (0.56)	18.1394
2010's	135 (76.27)	28.8240
2020's	41 (23.16)	9.53

Drug	Relationship Strength	Studies	Trials
alpha-ketoglutaramate	low	1	0
ammonium hydroxide	medium	53	8
betaine	low	1	0
butyric acid	low	1	0
carbamyl phosphate	low	3	0
carnitine	low	10	0
hippuric acid	low	1	0
n(g),n(g')-dimethyl-l-arginine	low	1	0
creatine	low	6	0
gamma-butyrobetaine	low	1	0
glycine	low	5	0
glycerol	medium	17	6
glycocyamine	low	3	0
hydrogen carbonate	low	1	0
kynurenine	low	1	0
pipecolic acid	low	1	0
nitrates	low	1	0
nitrites	low	1	0
orotic acid	low	5	0
phenylacetic acid	medium	8	2
uracil	low	1	0
uric acid	low	1	0
urea	medium	36	3
1-anilino-8-naphthalenesulfonate	medium	2	1
acetaminophen	low	1	0
clonidine	low	1	0
valproic acid	low	1	0
phenobarbital	low	1	0
4-phenylbutyric acid	medium	15	5
hydroxyproline	low	1	0
aspartic acid	low	3	0
glutamine	medium	25	7
lysine	low	2	0
uridine	low	1	0
methionine	low	2	0
ornithine	low	29	0
arginine	low	18	0
trimethylchlorosilane	low	1	0
isoprene	low	1	0
pyrimidine	low	1	0
citrulline	low	16	0
malondialdehyde	low	1	0
fluorescein-5-isothiocyanate	low	1	0
phenyl acetate	low	1	0
glutamic acid	low	2	0
s-adenosylmethionine	low	2	0
n-acetylaspartic acid	low	1	0
homocitrulline	low	3	0
n,n-bis(trimethylsilyl)-2,2,2-trifluoroacetamide	low	1	0
tyrosyltyrosine	low	1	0
phenylacetylglutamine	medium	9	4
valerates	low	1	0
carglumic acid	low	10	0
n,n-dimethylarginine	low	1	0
methylcyanocarbamate dimer	low	1	0
beta-methylcrotonylglycine	low	2	0
biotin	low	1	0
acetyl coenzyme a	low	1	0
sodium acetate, anhydrous	low	1	0
sodium benzoate	low	6	0
isomethyleugenol	low	1	0
sulfur	low	1	0
tiglyl-coenzyme a	low	1	0
glycerol phenylbutyrate	medium	17	5
s-adenosylmethionine	low	1	0
3-hydroxyisovalerylcarnitine	low	5	0
glutaminase	low	1	0
allopurinol	low	1	0

Protein Targets (505)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
Chain B, pheromone binding protein	0	0	1	1
Chain A, pheromone binding protein	0	0	1	1
Chain A, JmjC domain-containing histone demethylation protein 3A	2	0	0	2
Chain A, RNA-directed RNA polymerase NS5	0	1	0	1
acid sphingomyelinase	1	0	0	1
thioredoxin reductase	5	0	0	5
USP1 protein, partial	1	0	0	1
TDP1 protein	3	0	0	3
vitamin D3 receptor isoform VDRA	2	0	0	2
importin subunit beta-1 isoform 1	1	0	0	1
flap endonuclease 1	4	0	0	4
serine/threonine-protein kinase PLK1	1	0	0	1
snurportin-1	1	0	0	1
peptidyl-prolyl cis-trans isomerase NIMA-interacting 1	3	0	0	3
GTP-binding nuclear protein Ran isoform 1	1	0	0	1
DNA polymerase eta isoform 1	1	0	0	1
DNA polymerase iota isoform a (long)	4	0	0	4
urokinase-type plasminogen activator precursor	1	0	0	1
plasminogen precursor	1	0	0	1
urokinase plasminogen activator surface receptor precursor	1	0	0	1
geminin	6	0	0	6
DNA polymerase kappa isoform 1	2	0	0	2
fibroblast growth factor 22 isoform 1 precursor	0	0	0	1
Mitogen-activated protein kinase 13	0	1	0	1
Beta-lactamase	0	1	0	1
Transthyretin	0	0	1	1
Fatty acid-binding protein, intestinal	0	1	1	2
Fatty acid-binding protein, adipocyte	0	1	1	2
Cyclin-A2	0	1	0	1
Cannabinoid receptor 1	0	0	1	1
Cyclin-dependent kinase 2	0	1	1	2
Choline O-acetyltransferase	0	1	0	1
Mitogen-activated protein kinase 12	0	1	0	1
Guanine nucleotide-binding protein G	1	0	0	1
Fatty acid-binding protein 5	0	0	1	1
Fatty acid-binding protein 5	0	0	1	1
Mitogen-activated protein kinase 11	0	1	0	1
Mitogen-activated protein kinase 14	0	1	0	1
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	5	0	0	5
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain A, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
Chain B, AROMATIC AMINO ACID AMINOTRANSFERASE	0	0	1	1
mitogen-activated protein kinase 1	1	0	0	1
survival motor neuron protein isoform d	2	0	0	2
Histone deacetylase 3	0	6	0	6
Histone deacetylase 4	0	6	0	6
Histone deacetylase 1	0	6	0	6
Histone deacetylase 7	0	6	0	6
Histone deacetylase 2	0	6	0	6
Polyamine deacetylase HDAC10	0	4	0	4
Histone deacetylase 11	0	4	0	4
Histone deacetylase 8	0	6	0	6
NAD-dependent protein deacylase sirtuin-5, mitochondrial	0	2	0	2
Histone deacetylase 6	0	6	0	6
Histone deacetylase 9	0	6	0	6
Histone deacetylase 5	0	6	0	6
RAR-related orphan receptor gamma	7	0	0	7
ATAD5 protein, partial	1	0	0	1
GLS protein	4	0	0	4
Microtubule-associated protein tau	2	0	0	2
Smad3	2	0	0	2
aldehyde dehydrogenase 1 family, member A1	7	0	0	7
thyroid stimulating hormone receptor	5	0	0	5
nuclear receptor subfamily 1, group I, member 3	6	0	0	6
progesterone receptor	5	0	0	5
EWS/FLI fusion protein	1	0	0	1
retinoic acid nuclear receptor alpha variant 1	8	0	0	8
retinoid X nuclear receptor alpha	5	0	0	5
estrogen-related nuclear receptor alpha	9	0	0	9
estrogen nuclear receptor alpha	12	0	0	12
peroxisome proliferator activated receptor gamma	5	0	0	5
aryl hydrocarbon receptor	2	0	0	2
chromobox protein homolog 1	3	0	0	3
thyroid hormone receptor beta isoform 2	4	0	0	4
peripheral myelin protein 22	1	0	0	1
cytochrome P450 3A4 isoform 1	1	0	0	1
Gamma-aminobutyric acid receptor subunit pi	1	1	0	2
ATP-binding cassette sub-family C member 3	0	2	0	2
Multidrug resistance-associated protein 4	0	2	0	2
Solute carrier family 22 member 6	0	2	0	3
Carbonic anhydrase 12	0	1	0	1
UDP-glucuronosyltransferase 1A9	0	0	0	2
Bile salt export pump	0	3	0	3
Voltage-dependent calcium channel gamma-2 subunit	3	0	0	3
Bile salt export pump	0	6	0	6
Cytochrome P450 2B1	0	0	0	1
Cytochrome P450 1A1	0	0	0	1
Carbonic anhydrase 1	0	6	0	9
Carbonic anhydrase 2	0	6	0	9
Myoglobin	0	1	0	1
Cytochrome P450 1A1	0	0	1	2
Cytochrome P450 1A2	0	0	0	1
Prostaglandin G/H synthase 1	0	1	0	1
Carbonic anhydrase 3	0	1	0	1
Cytochrome P450 3A4	0	1	0	2
Cytochrome P450 2D6	0	1	0	1
Cytochrome P450 2C9	0	1	0	1
Polyunsaturated fatty acid lipoxygenase ALOX15	0	1	0	1
Gamma-aminobutyric acid receptor subunit beta-1	1	1	0	2
Sulfotransferase 1A1	0	0	0	1
Gamma-aminobutyric acid receptor subunit delta	1	1	0	2
Gamma-aminobutyric acid receptor subunit gamma-2	1	1	0	2
UDP-glucuronosyltransferase 1-6	0	0	0	2
Glutamate receptor 2	3	2	1	6
Gamma-aminobutyric acid receptor subunit alpha-5	1	1	0	2
Gamma-aminobutyric acid receptor subunit alpha-3	1	1	0	2
Arachidonate 5-lipoxygenase-activating protein	0	1	0	1
UDP-glucuronosyltransferase 1A1	0	0	0	1
Carbonic anhydrase 4	0	7	0	7
Prostaglandin G/H synthase 1	0	1	0	1
Carbonic anhydrase 6	0	1	0	1
Gamma-aminobutyric acid receptor subunit gamma-1	1	1	0	2
Gamma-aminobutyric acid receptor subunit alpha-2	1	1	0	2
Gamma-aminobutyric acid receptor subunit alpha-4	1	1	0	2
Gamma-aminobutyric acid receptor subunit gamma-3	1	1	0	2
Gamma-aminobutyric acid receptor subunit alpha-6	1	1	0	2
Carbonic anhydrase 5A, mitochondrial	0	3	0	3
Prostaglandin G/H synthase 2	0	0	0	1
Carbonic anhydrase 7	0	1	0	1
Nuclear receptor ROR-gamma	2	0	0	2
Cytochrome P450 2J2	0	1	0	1
Gamma-aminobutyric acid receptor subunit alpha-1	1	1	0	2
Gamma-aminobutyric acid receptor subunit beta-3	1	1	0	2
Gamma-aminobutyric acid receptor subunit beta-2	1	1	0	2
Carbonic anhydrase 9	0	3	0	3
GABA theta subunit	1	1	0	2
Canalicular multispecific organic anion transporter 1	0	2	0	2
Carbonic anhydrase 15	0	4	0	4
Carbonic anhydrase 13	0	1	0	1
Gamma-aminobutyric acid receptor subunit epsilon	1	1	0	2
Carbonic anhydrase 14	0	1	0	1
Carbonic anhydrase 5B, mitochondrial	0	1	0	1
toxin B	0	0	0	1
N-alpha-acetyltransferase 50	0	0	1	1
interleukin 8	2	0	0	2
AR protein	11	0	0	11
estrogen receptor 2 (ER beta)	2	0	0	2
glucocorticoid receptor [Homo sapiens]	4	0	0	4
IDH1	1	0	0	1
euchromatic histone-lysine N-methyltransferase 2	6	0	0	6
Bloom syndrome protein isoform 1	3	0	0	3
transcriptional regulator ERG isoform 3	2	0	0	2
ras-related protein Rab-9A	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 1	8	0	0	8
lamin isoform A-delta10	7	0	0	7
Polyphenol oxidase 2	0	1	0	1
Hypoxanthine-guanine phosphoribosyltransferase	0	0	0	1
Cellular tumor antigen p53	7	0	0	7
Xanthine dehydrogenase/oxidase [Includes: Xanthine dehydrogenase	0	1	0	1
Adenosine receptor A2a	0	0	1	1
Xanthine dehydrogenase/oxidase	0	2	0	2
Nuclear receptor ROR-gamma	0	1	0	1
Xanthine dehydrogenase/oxidase	0	2	1	4
Purine nucleoside phosphorylase	0	0	1	1
TAR DNA-binding protein 43	2	0	0	2
Shiga toxin subunit A	0	1	0	1
Histamine H3 receptor	0	1	0	1
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	2	2
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	2	2
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	2	2
phosphopantetheinyl transferase	6	0	0	6
NFKB1 protein, partial	2	0	0	2
alpha-galactosidase	1	0	0	1
cytochrome P450 2C19 precursor	4	0	0	4
muscarinic acetylcholine receptor M1	2	0	0	2
lethal factor (plasmid)	3	0	0	3
Nitric oxide synthase, endothelial	0	0	0	2
Nitric oxide synthase, brain	0	2	0	3
Nitric oxide synthase, brain	0	2	0	3
Nitric oxide synthase, inducible	0	0	0	1
Nitric oxide synthase, inducible	0	0	1	3
Cationic amino acid transporter 3	0	2	0	2
ATP-dependent phosphofructokinase	2	0	0	2
Chain A, TYROSYL-DNA PHOSPHODIESTERASE	3	0	0	3
Chain A, Ferritin light chain	2	0	0	2
arylsulfatase A	4	0	0	4
Metabotropic glutamate receptor 6	0	1	2	4
Excitatory amino acid transporter 4	0	2	0	2
Glutamate transporter homolog	0	0	1	1
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase	0	1	0	1
5-hydroxytryptamine receptor 1D	0	0	0	1
Glutamate receptor ionotropic, NMDA 1	0	5	2	7
Excitatory amino acid transporter 1	0	3	0	4
Excitatory amino acid transporter 2	0	3	0	4
Excitatory amino acid transporter 3	0	3	0	4
Glutamate receptor ionotropic, NMDA 2A	0	5	2	7
Glutamate receptor ionotropic, NMDA 2B	0	5	2	7
Glutamate receptor ionotropic, NMDA 2C	0	5	2	7
Metabotropic glutamate receptor 1	0	1	2	4
Metabotropic glutamate receptor 2	0	1	2	4
Carbonic anhydrase-like protein, putative	0	0	0	3
Glutamate receptor ionotropic, NMDA 2D	0	5	2	7
Glutamate receptor ionotropic, NMDA 3B	0	5	2	7
Carbonic anhydrase 4	0	0	0	1
Glutamate receptor ionotropic, NMDA 3A	0	5	2	7
Chain A, Betaine ABC transporter permease and substrate binding protein	0	0	1	1
Chain A, Osmoprotection protein (ProX)	0	0	1	1
Chain A, Glycine betaine/carnitine/choline-binding protein	0	0	1	1
Chain A, Glycine betaine/carnitine/choline-binding protein	0	0	1	1
Chain A, Glycine betaine/carnitine/choline-binding protein	0	0	1	1
Chain A, Glycine betaine/carnitine/choline-binding protein	0	0	1	1
GLI family zinc finger 3	7	0	0	7
Proton-coupled amino acid transporter 1	0	7	0	8
Chain A, Avidin	0	0	1	1
Chain A, Protein (streptavidin)	0	0	1	1
Chain B, Protein (streptavidin)	0	0	1	1
Chain A, Streptavidin	0	0	1	1
Chain D, Streptavidin	0	0	1	1
Chain A, Streptavidin	0	0	1	1
Chain D, Streptavidin	0	0	1	1
Chain A, Streptavidin	0	0	1	1
Chain D, Streptavidin	0	0	1	1
Chain A, Streptavidin Complex With Biotin	0	0	1	1
Chain D, Circularly Permuted Core-streptavidin E51/a46	0	0	1	1
Chain A, Core-streptavidin	0	0	1	1
Chain D, Core-streptavidin	0	0	1	1
Chain A, Core-streptavidin	0	0	1	1
Chain D, Core-streptavidin	0	0	1	1
Chain A, Core-streptavidin	0	0	1	1
Chain D, Core-streptavidin	0	0	1	1
Chain A, Core-streptavidin	0	0	1	1
Chain D, Core-streptavidin	0	0	1	1
Chain A, Avidin	0	0	1	1
Chain A, Streptavidin	0	0	1	1
Chain B, Streptavidin	0	0	1	1
Chain A, Streptavidin	0	0	1	1
Chain B, Streptavidin	0	0	1	1
apical membrane antigen 1, AMA1	1	0	0	1
nonstructural protein 1	0	1	0	1
green fluorescent protein, partial	0	1	0	1
insulin-degrading enzyme isoform 1	0	0	1	1
Streptavidin	0	0	1	1
Receptor-type tyrosine-protein phosphatase beta	0	0	0	1
Chain A, Nitrile Hydratase alpha subunit	0	1	0	1
Chain B, Nitrile Hydratase beta subunit	0	1	0	1
SMAD family member 2	1	0	0	1
SMAD family member 3	1	0	0	1
Lysine-specific demethylase 4E	0	1	0	1
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a	3	0	0	3
thyroid stimulating hormone receptor	2	0	0	2
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a	3	0	0	3
Free fatty acid receptor 3	0	0	1	1
Solute carrier family 22 member 20	0	3	0	3
Solute carrier family 22 member 6	0	3	0	3
Methyl-accepting chemotaxis protein NahY	0	0	1	1
GALC protein	1	0	0	1
Solute carrier family 22 member 5	0	0	0	1
Solute carrier family 22 member 5	0	0	0	1
Solute carrier family 22 member 16	0	0	0	1
Solute carrier family 22 member 21	0	0	0	1
Solute carrier family 22 member 5	0	0	0	1
N(G),N(G)-dimethylarginine dimethylaminohydrolase 1	0	2	0	3
General amino-acid permease GAP1	0	0	0	1
Chain A, Cruzipain	1	0	0	1
acetylcholinesterase	2	0	0	2
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)	1	0	0	1
regulator of G-protein signaling 4	2	0	0	2
cytochrome P450 2D6	2	0	0	2
Beta-1 adrenergic receptor	0	0	1	1
Alpha-1B adrenergic receptor	0	0	1	1
cytochrome P450 2D6 isoform 1	1	0	0	1
cytochrome P450 2C9 precursor	1	0	0	1
Membrane primary amine oxidase	0	1	0	1
Solute carrier family 22 member 1	0	2	0	3
5-hydroxytryptamine receptor 3A	0	0	1	1
Solute carrier family 22 member 3	0	2	0	2
5-hydroxytryptamine receptor 1A	0	1	0	1
Alpha-2A adrenergic receptor	0	2	1	3
Alpha-1B adrenergic receptor	0	1	0	1
Alpha-2B adrenergic receptor	0	2	1	3
Alpha-1A adrenergic receptor	0	0	1	1
Alpha-2C adrenergic receptor	0	2	1	3
D	0	1	0	1
D(3) dopamine receptor	0	1	0	1
5-hydroxytryptamine receptor 1A	0	2	0	2
Alpha-2B adrenergic receptor	0	2	0	2
Amine oxidase [flavin-containing] A	0	1	0	1
Alpha-2C adrenergic receptor	0	2	0	2
Alpha-2A adrenergic receptor	0	2	0	2
Alpha-1D adrenergic receptor	0	1	0	1
Alpha-1D adrenergic receptor	0	2	1	3
D(1B) dopamine receptor	0	1	0	1
Amine oxidase [flavin-containing] B	0	1	0	1
5-hydroxytryptamine receptor 1B	0	2	0	2
Adenosine receptor A2b	0	0	1	1
Adenosine receptor A2a	0	0	1	1
Alpha-2B adrenergic receptor	0	0	0	1
D(4) dopamine receptor	0	1	0	1
Delta-type opioid receptor	0	1	0	1
Alpha-1A adrenergic receptor	0	1	1	2
Alpha-1B adrenergic receptor	0	1	1	2
Alpha-1A adrenergic receptor	0	1	1	2
Histamine H2 receptor	1	0	0	1
5-hydroxytryptamine receptor 7	0	1	0	1
Spike glycoprotein	1	0	0	1
D(2) dopamine receptor	0	1	0	1
Mu-type opioid receptor	0	2	0	2
Inositol monophosphatase 1	1	0	0	1
Alpha-2C adrenergic receptor	0	0	0	1
Alpha-2A adrenergic receptor	0	0	0	1
Alpha-2A adrenergic receptor	0	1	0	1
Nischarin	0	2	0	2
Solute carrier family 22 member 1	0	1	0	1
Multidrug and toxin extrusion protein 1	0	1	0	1
Nischarin	0	1	0	1
Sodium- and chloride-dependent creatine transporter 1	0	1	0	1
hypoxia-inducible factor 1 alpha subunit	1	0	0	1
peroxisome proliferator-activated receptor delta	4	0	0	4
vitamin D (1,25- dihydroxyvitamin D3) receptor	1	0	0	1
activating transcription factor 6	3	0	0	3
thyrotropin-releasing hormone receptor	1	0	0	1
Histone H2A.x	2	0	0	2
heat shock protein beta-1	2	0	0	2
Chain A, GLUTAMATE RECEPTOR SUBUNIT 2	0	1	0	1
Chain A, Glutamate Receptor Subunit 2	0	1	0	1
Chain B, Glutamate Receptor Subunit 2	0	1	0	1
Chain A, Slr1257 protein	0	0	1	1
Chain A, Glucosamine--fructose-6-phosphate aminotransferase [isomerizing]	0	1	0	1
glp-1 receptor, partial	1	0	0	1
beta-2 adrenergic receptor	1	0	0	1
Metabotropic glutamate receptor 8	0	2	0	2
Glutamate receptor ionotropic, NMDA 2D	0	1	0	1
Glutamate receptor ionotropic, NMDA 3B	0	1	0	1
Glutathione reductase, mitochondrial	0	4	0	4
Bifunctional aspartokinase/homoserine dehydrogenase 1	0	1	0	1
Prothrombin	0	0	1	1
Integrin beta-3	1	0	1	2
Integrin alpha-IIb	1	0	1	2
ATP-citrate synthase	0	1	0	1
Glutamate receptor 1	0	2	1	3
Glutamate receptor 3	0	2	1	3
Glutamate receptor 4	0	2	1	3
Glutamate receptor ionotropic, kainate 1	0	2	1	3
Metabotropic glutamate receptor 1	0	1	1	2
Metabotropic glutamate receptor 2	0	0	1	1
Metabotropic glutamate receptor 3	0	0	1	1
Metabotropic glutamate receptor 4	0	1	1	2
Metabotropic glutamate receptor 5	0	0	1	1
Metabotropic glutamate receptor 6	0	0	1	1
Metabotropic glutamate receptor 7	0	0	1	1
Glutamate receptor ionotropic, kainate 1	0	1	1	2
Metabotropic glutamate receptor 5	0	1	1	3
Glutamate receptor ionotropic, kainate 2	0	2	1	3
Glutamate receptor 1	0	2	1	3
Glutamate receptor 2	0	2	1	3
Glutamate receptor 3	0	1	1	2
Glutamate receptor ionotropic, kainate 3	0	2	0	2
Metabotropic glutamate receptor 8	0	0	1	1
Glutamate receptor 4	0	2	1	3
Excitatory amino acid transporter 3	0	0	0	1
Glutamate racemase	0	0	0	1
Metabotropic glutamate receptor 8	0	1	1	2
Fatty-acid amide hydrolase 1	0	1	0	1
Glutamate receptor ionotropic, kainate 4	0	2	0	2
Glutamate carboxypeptidase 2	0	1	0	1
Glutamate receptor ionotropic, NMDA 1	0	1	0	1
Glutamate receptor ionotropic, NMDA 2A	0	1	0	1
Glutamate receptor ionotropic, kainate 2	0	1	1	2
Glutamate receptor ionotropic, kainate 3	0	1	0	1
Glutamate receptor ionotropic, NMDA 2B	0	1	0	1
Metabotropic glutamate receptor 7	0	1	1	3
Metabotropic glutamate receptor 3	0	1	1	3
Metabotropic glutamate receptor 4	0	1	1	3
Glutamate receptor ionotropic, NMDA 2C	0	1	0	1
Glutamate receptor ionotropic, kainate 5	0	1	0	1
Glutamate receptor ionotropic, kainate 5	0	2	0	2
Glutamate racemase	0	0	0	1
Prolyl 4-hydroxylase	0	1	0	1
Glutamate receptor ionotropic, NMDA 3A	0	1	0	1
Alpha-ketoglutarate-dependent dioxygenase FTO	0	1	0	1
Protein-glutamine gamma-glutamyltransferase 2	0	1	0	1
Chain A, Glutamine Binding Protein	0	0	1	1
15-lipoxygenase, partial	1	0	0	1
peripheral myelin protein 22 isoform 1	1	0	0	1
histone-lysine N-methyltransferase 2A isoform 2 precursor	1	0	0	1
Neutral amino acid transporter A	0	2	0	2
Asc-type amino acid transporter 1	0	2	0	2
Neutral amino acid transporter B(0)	0	2	0	2
Amino acid transporter	0	2	0	2
Chain B, EUKARYOTIC TRANSLATION INITIATION FACTOR 4E	0	0	1	1
thyroid hormone receptor beta isoform a	2	0	0	2
Glycine receptor subunit alpha-1	0	0	1	1
Sodium- and chloride-dependent GABA transporter 2	0	1	0	1
Sodium- and chloride-dependent GABA transporter 3	0	1	0	1
Sodium- and chloride-dependent glycine transporter 1	0	1	0	1
Large neutral amino acids transporter small subunit 1	0	5	0	5
Serine racemase	0	1	0	1
Olfactory receptor 51E2	0	0	2	2
Sodium- and chloride-dependent glycine transporter 2	0	1	0	1
pregnane X nuclear receptor	2	0	0	2
Dihydrofolate reductase	0	1	0	1
Aldo-keto reductase family 1 member B1	0	2	0	2
Solute carrier family 22 member 6	0	2	0	4
Solute carrier family 22 member 8	0	2	0	2
Solute carrier family 22 member 8	0	1	0	1
Carbonic anhydrase	0	3	0	3
cellular tumor antigen p53 isoform a	1	0	0	1
Chain A, Putative fructose-1,6-bisphosphate aldolase	1	0	0	1
Chain A, 2-oxoglutarate Oxygenase	1	0	0	1
nuclear receptor ROR-gamma isoform 1	1	0	0	1
Chain A, AMINOPEPTIDASE	0	1	0	1
Chain A, AMINOPEPTIDASE	0	1	0	1
Chain A, Leucine Aminopeptidase	0	1	0	1
Alkaline phosphatase, tissue-nonspecific isozyme	0	1	0	1
Intestinal-type alkaline phosphatase	0	1	0	1
Phospholipase A-2-activating protein	0	1	0	1
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast	0	1	0	1
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast	0	1	0	1
potassium voltage-gated channel subfamily H member 2 isoform d	2	0	0	2
Chain A, Methionyl-tRNA synthetase	0	0	1	1
Chain A, Methionyl-tRNA synthetase	0	0	1	1
Chain A, Aminopeptidase	0	1	0	1
Chain A, Methionine aminopeptidase	0	1	0	1
S-ribosylhomocysteine lyase	0	1	0	1
Adenylate cyclase type 5	0	0	0	1
S-adenosylmethionine synthase isoform type-1	0	0	0	1
S-adenosylmethionine synthase isoform type-2	0	0	0	1
Fatty acid synthase	0	1	0	1
67.9K protein	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 2	1	0	0	1
Rap guanine nucleotide exchange factor 4	1	0	0	1
N(G),N(G)-dimethylarginine dimethylaminohydrolase 1	0	0	0	1
N(G),N(G)-dimethylarginine dimethylaminohydrolase 1	0	0	0	1
Sodium/iodide cotransporter	0	1	0	1
Chain A, ARGINASE 1	0	1	0	1
Chain A, Arginase 1	0	1	0	1
Chain A, ARGINASE 1	0	1	0	1
Chain A, L-ARGININE\\:GLYCINE AMIDINOTRANSFERASE	0	1	0	1
v-jun sarcoma virus 17 oncogene homolog (avian)	3	0	0	3
Solute carrier family 2, facilitated glucose transporter member 9	0	2	2	4
Sodium channel protein type 1 subunit alpha	0	1	0	1
Potassium voltage-gated channel subfamily H member 2	0	1	0	1
Sodium channel protein type 2 subunit alpha	0	1	0	1
Sodium channel protein type 3 subunit alpha	0	1	0	1
Olfactory receptor class A-like protein 1	0	0	1	1
Tyrosine-protein kinase Lck	0	0	1	1
Aldo-keto reductase family 1 member B1	0	2	0	2
Prolyl 4-hydroxylase subunit alpha-1	0	1	0	1
Peroxisomal sarcosine oxidase	0	0	0	1
Met repressor	0	0	2	2
Protein arginine N-methyltransferase 5	0	0	1	1
Histone H3K27 methylase	0	0	0	1
Phenylethanolamine N-methyltransferase	0	0	1	2
S-adenosylmethionine decarboxylase proenzyme	0	1	0	2
Protein-L-isoaspartate(D-aspartate) O-methyltransferase	0	1	0	1
Catechol O-methyltransferase	0	0	0	1
Protein arginine N-methyltransferase 1	0	1	0	1
Methylosome protein 50	0	0	1	1
Free fatty acid receptor 3	0	0	1	1
Carbonic anhydrase	0	2	0	2
Solute carrier family 15 member 1	0	1	1	2
Tyrosine--tRNA ligase, cytoplasmic	0	1	0	1
Chain A, Uracil-DNA Glycosylase	0	1	0	1
Protein cereblon	0	4	0	4
caspase 7, apoptosis-related cysteine protease	1	0	0	1
caspase-3	1	0	0	1
Rap guanine nucleotide exchange factor 3	2	0	0	2
Urease subunit alpha	0	1	0	1
Urease subunit beta	0	1	0	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Guanine deaminase	0	1	0	1
Chain A, Cytidine Deaminase	0	1	0	1
nonstructural protein 1	1	0	0	1
Sodium/nucleoside cotransporter 1	0	2	0	2
Sodium/nucleoside cotransporter 2	0	2	0	2
Cytidine deaminase	0	1	0	1
Equilibrative nucleoside transporter 2	0	2	0	2
Equilibrative nucleoside transporter 1	0	2	0	2
Uridine-cytidine kinase 1	0	0	0	1
Solute carrier family 28 member 3	0	2	0	2
Carbonic anhydrase	0	1	0	1
Chain A, Arginase 1	0	1	0	1
Luciferase	1	0	0	1
pregnane X receptor	1	0	0	1
farnesoid X nuclear receptor	1	0	0	1
Cocaine esterase	0	1	0	1
Cytochrome P450 2A6	0	1	0	1
Tissue factor	0	1	0	1
Aldo-keto reductase family 1 member A1	0	1	0	1
UDP-glucuronosyltransferase 2B7	0	1	0	2
Liver carboxylesterase 1	0	1	0	1
Aldo-keto reductase family 1 member A1	0	1	0	1
Aldo-keto reductase family 1 member A1	0	1	0	1
Aldo-keto reductase family 1 member B7	0	1	0	1
Sigma non-opioid intracellular receptor 1	0	1	0	1
ATPase family AAA domain-containing protein 5	1	0	0	1
Ataxin-2	1	0	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Synonyms(2)

Research Excerpts

Overview

Treatment

Research

Studies (177)

Protein Targets (505)

Inborn Urea Cycle Disorder

Synonyms(2)

Research Excerpts

Overview

Treatment

Research

Studies (177)

Drugs Studied (77)

Protein Targets (505)