Argininosuccinic Aciduria

Rare autosomal recessive disorder of the urea cycle which leads to the accumulation of argininosuccinic acid in body fluids and severe HYPERAMMONEMIA. Clinical features of the neonatal onset of the disorder include poor feeding, vomiting, lethargy, seizures, tachypnea, coma, and death. Later onset results in milder set of clinical features including vomiting, failure to thrive, irritability, behavioral problems, or psychomotor retardation. Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder.

Synonym
Argininosuccinic Aciduria

Excerpt	Reference
"Argininosuccinic aciduria is an inborn error of the urea cycle caused by deficiency of argininosuccinate lyase (ASL)."	( Chen, BC; Ngu, LH; Zabedah, MY, 2010)
"Argininosuccinic aciduria is an autosomal, recessive amino acid disorder that is caused by a deficiency of the argininosuccinate lyase enzyme."	( Sahi, N; Salmanizadeh, H, 2020)
"Argininosuccinic aciduria is a genetic disorder of the urea cycle caused by a deficiency of argininosuccinate lyase."	( Kawano, H; Mitsubuchi, H; Morikawa, K; Nagano, H; Nagayoshi, Y; Nakayama, M; Nishi, M; Nishihara, T; Sakaino, N; Tsujita, K, 2023)
"Epilepsy in argininosuccinic aciduria is frequent, polymorphic, and associated with more frequent neurodevelopmental comorbidities."	( Baruteau, J; Bernhardt, I; Boyd, S; Chakrapani, A; Chan, A; Cleary, M; Crowther, L; Dionisi-Vici, C; Elkhateeb, N; Gissen, P; Grunewald, S; Hartley, T; Lachmann, R; Mills, P; Morris, AAM; Mundy, H; Murphy, E; Olivieri, G; Ridout, D; Santra, S; Sharma, R; Siri, B; Stepien, KM; Sudakhar, S; Uudelepp, ML; Yeo, M, 2023)

Excerpt	Reference
"He had been treated for argininosuccinic aciduria since 8 months of age."	( Kawano, H; Mitsubuchi, H; Morikawa, K; Nagano, H; Nagayoshi, Y; Nakayama, M; Nishi, M; Nishihara, T; Sakaino, N; Tsujita, K, 2023)

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	55 (45.83)	23.3326
1990's	15 (12.50)	12.5806
2000's	16 (13.33)	18.1394
2010's	25 (20.83)	28.8240
2020's	9 (7.50)	9.53

Drug	Relationship Strength	Studies	Trials
adipic acid	low	1	0
ammonium hydroxide	medium	43	1
benzoic acid	low	1	0
carbamates	low	1	0
carbamyl phosphate	low	1	0
citric acid, anhydrous	low	2	0
hydrogen sulfide	low	1	0
creatine	low	3	0
lactic acid	low	1	0
dimethyl sulfoxide	low	1	0
glutaric acid	low	1	0
glycine	low	3	0
glycerol	low	1	0
glycocyamine	low	2	0
methanol	low	1	0
nitric acid	medium	1	1
nitrites	low	1	0
orotic acid	low	3	0
pteridines	low	1	0
uric acid	low	1	0
4-phenylbutyric acid	low	1	0
alanine	low	3	0
aspartic acid	low	2	0
glutamine	low	5	0
lysine	low	2	0
leucine	low	2	0
fluocinolone acetonide	low	1	0
ornithine	low	8	0
histidine	low	1	0
acetonitrile	low	1	0
citrulline	low	26	0
isovaleric acid	low	1	0
molybdenum	low	1	0
tetradecanoylphorbol acetate	low	1	0
transferrin	low	1	0
potassium phosphate	low	1	0
phenylacetylglutamine	low	1	0
phorbols	low	1	0
atropine	low	1	0
mevalonic acid	low	1	0
tretinoin	low	1	0
sodium benzoate	low	3	0
vitamin k semiquinone radical	low	1	0
fumarates	low	1	0
glycerol phenylbutyrate	low	1	0
exudates	low	1	0
cyclic gmp	low	1	0
molybdenum cofactor	low	1	0

Protein Targets (418)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
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	2	0	0	2
survival motor neuron protein isoform d	2	0	0	2
Histone deacetylase 3	0	2	0	2
Histone deacetylase 4	0	2	0	2
Histone deacetylase 1	0	2	0	2
Histone deacetylase 7	0	2	0	2
Histone deacetylase 2	0	2	0	2
Polyamine deacetylase HDAC10	0	1	0	1
Histone deacetylase 11	0	1	0	1
Histone deacetylase 8	0	2	0	2
NAD-dependent protein deacylase sirtuin-5, mitochondrial	0	1	0	1
Histone deacetylase 6	0	2	0	2
Histone deacetylase 9	0	2	0	2
Histone deacetylase 5	0	2	0	2
pregnane X receptor	2	0	0	2
retinoic acid nuclear receptor alpha variant 1	11	0	0	11
nuclear factor erythroid 2-related factor 2 isoform 1	8	0	0	8
RAR-related orphan receptor gamma	6	0	0	6
GLI family zinc finger 3	10	0	0	10
AR protein	8	0	0	8
cytochrome P450 family 3 subfamily A polypeptide 4	4	0	0	4
glucocorticoid receptor [Homo sapiens]	7	0	0	7
estrogen nuclear receptor alpha	9	0	0	9
cytochrome P450 2D6	2	0	0	2
peroxisome proliferator-activated receptor delta	4	0	0	4
thyroid hormone receptor beta isoform a	4	0	0	4
histone acetyltransferase KAT2A isoform 1	4	0	0	4
lethal factor (plasmid)	5	0	0	5
lamin isoform A-delta10	6	0	0	6
Cellular tumor antigen p53	6	0	0	6
Solute carrier family 22 member 20	0	4	0	4
Solute carrier family 22 member 6	0	4	0	4
Chain A, TYROSYL-DNA PHOSPHODIESTERASE	4	0	0	4
Carbonic anhydrase 1	0	4	0	9
Carbonic anhydrase 2	0	4	0	9
Alanine racemase, biosynthetic	0	0	0	1
5-hydroxytryptamine receptor 1D	0	0	0	3
Glutamate receptor ionotropic, NMDA 1	0	4	1	6
Proton-coupled amino acid transporter 1	0	5	0	6
Adenosine deaminase	0	0	0	1
Carbonic anhydrase 4	0	0	0	3
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	3	3
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	3	3
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN	0	0	3	3
thioredoxin reductase	4	0	0	4
phosphopantetheinyl transferase	4	0	0	4
NFKB1 protein, partial	1	0	0	1
GLS protein	3	0	0	3
thyroid stimulating hormone receptor	3	0	0	3
alpha-galactosidase	1	0	0	1
euchromatic histone-lysine N-methyltransferase 2	6	0	0	6
cytochrome P450 2C19 precursor	2	0	0	2
chromobox protein homolog 1	4	0	0	4
flap endonuclease 1	3	0	0	3
DNA polymerase iota isoform a (long)	4	0	0	4
muscarinic acetylcholine receptor M1	1	0	0	1
Cytochrome P450 3A4	0	0	1	2
Nitric oxide synthase, endothelial	0	0	0	2
Nitric oxide synthase, brain	0	1	0	2
Nitric oxide synthase, brain	0	1	0	2
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	3	0	0	3
Chain A, Ferritin light chain	3	0	0	3
aldehyde dehydrogenase 1 family, member A1	6	0	0	6
arylsulfatase A	2	0	0	2
Bloom syndrome protein isoform 1	3	0	0	3
Metabotropic glutamate receptor 6	0	0	1	1
Excitatory amino acid transporter 4	0	1	0	1
Glutamate transporter homolog	0	0	1	1
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase	0	1	0	1
Excitatory amino acid transporter 1	0	1	0	1
Excitatory amino acid transporter 2	0	1	0	1
Excitatory amino acid transporter 3	0	1	0	1
Glutamate receptor ionotropic, NMDA 2A	0	3	1	5
Glutamate receptor ionotropic, NMDA 2B	0	3	1	5
Glutamate receptor ionotropic, NMDA 2C	0	3	1	5
Metabotropic glutamate receptor 1	0	0	1	1
Metabotropic glutamate receptor 2	0	0	1	1
Carbonic anhydrase-like protein, putative	0	0	0	3
Glutamate receptor ionotropic, NMDA 2D	0	3	1	5
Glutamate receptor ionotropic, NMDA 3B	0	3	1	5
Glutamate receptor ionotropic, NMDA 3A	0	3	1	5
Chain A, 2-oxoglutarate Oxygenase	3	0	0	3
nuclear receptor subfamily 1, group I, member 3	7	0	0	7
retinoid X nuclear receptor alpha	7	0	0	7
pregnane X nuclear receptor	5	0	0	5
cystic fibrosis transmembrane conductance regulator	0	1	0	1
DNA polymerase beta	1	0	0	1
short transient receptor potential channel 6 isoform 1	0	0	1	1
Solute carrier family 22 member 1	0	1	0	1
ATP-binding cassette sub-family C member 3	0	2	0	2
Multidrug resistance-associated protein 4	0	2	0	3
5-hydroxytryptamine receptor 4	0	2	0	2
Bile salt export pump	0	3	0	3
Muscarinic acetylcholine receptor M2	0	2	1	6
Muscarinic acetylcholine receptor M4	0	2	1	5
Muscarinic acetylcholine receptor M1	0	2	1	5
Muscarinic acetylcholine receptor M3	0	2	1	5
Muscarinic acetylcholine receptor M4	0	2	1	5
Muscarinic acetylcholine receptor M5	0	2	1	5
Muscarinic acetylcholine receptor M5	0	2	1	5
Muscarinic acetylcholine receptor M2	0	2	1	6
Muscarinic acetylcholine receptor M1	0	2	1	6
Muscarinic acetylcholine receptor M1	0	0	0	1
Muscarinic acetylcholine receptor M3	0	2	1	5
Alpha-1D adrenergic receptor	0	2	0	2
5-hydroxytryptamine receptor 2C	0	2	0	2
Muscarinic acetylcholine receptor M4	0	1	0	1
Histamine H2 receptor	0	0	0	1
5-hydroxytryptamine receptor 7	0	1	0	1
Reverse transcriptase/RNaseH	0	0	0	1
Muscarinic acetylcholine receptor	0	1	1	2
Canalicular multispecific organic anion transporter 1	0	2	0	2
Chain A, Oxygen-insensitive Nad(p)h Nitroreductase	0	1	0	1
Chain B, Oxygen-insensitive Nad(p)h Nitroreductase	0	1	0	1
Luciferase	2	0	0	2
acetylcholinesterase	3	0	0	3
estrogen receptor 2 (ER beta)	5	0	0	5
estrogen-related nuclear receptor alpha	8	0	0	8
Polyphenol oxidase 2	0	1	0	1
D-amino-acid oxidase	0	0	1	1
Neuronal acetylcholine receptor subunit alpha-4	0	1	0	1
Neuronal acetylcholine receptor subunit beta-2	0	1	0	1
Tyrosinase	0	1	0	1
D-amino-acid oxidase	0	2	1	3
Metabotropic glutamate receptor 5	0	0	1	1
Alpha-glucosidase MAL12	0	1	0	1
Monocarboxylate transporter 1	0	0	0	1
Beta-lactamase	0	1	1	2
Nicotinate phosphoribosyltransferase	0	1	0	1
D-aspartate oxidase	0	1	0	1
Serine racemase	0	2	0	2
Carbonic anhydrase 4	0	5	0	6
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, Proto-oncogene Tyrosine-protein Kinase Src	0	1	0	1
Chain A, N5-carboxyaminoimidazole ribonucleotide mutase	0	0	1	1
Chain B, N5-carboxyaminoimidazole ribonucleotide mutase	0	0	1	1
Chain A, N5-carboxyaminoimidazole ribonucleotide mutase	0	0	1	1
Chain B, N5-carboxyaminoimidazole ribonucleotide mutase	0	0	1	1
Chain A, JmjC domain-containing histone demethylation protein 3A	1	0	0	1
farnesoid X nuclear receptor	2	0	0	2
peroxisome proliferator activated receptor gamma	3	0	0	3
hepatocyte nuclear factor 4-alpha isoform 2	0	1	0	1
perilipin-5	0	1	0	1
perilipin-1	0	1	0	1
1-acylglycerol-3-phosphate O-acyltransferase ABHD5 isoform a	0	1	0	1
Beta-lactamase	0	1	0	1
Proto-oncogene tyrosine-protein kinase Src	0	1	0	1
3-dehydroquinate dehydratase	0	0	1	1
ATP-citrate synthase	0	0	0	1
Ribonuclease T	0	0	0	1
Cell death-related nuclease 4	0	0	0	1
3-dehydroquinate dehydratase	0	0	1	1
Prolyl 4-hydroxylase	0	1	0	1
Alpha-ketoglutarate-dependent dioxygenase FTO	0	1	0	1
N(G),N(G)-dimethylarginine dimethylaminohydrolase 1	0	1	0	1
General amino-acid permease GAP1	0	0	0	1
Sodium- and chloride-dependent creatine transporter 1	0	1	0	1
Phosphodiesterase	0	1	0	1
Multidrug resistance-associated protein 5	0	0	0	1
cGMP-inhibited 3',5'-cyclic phosphodiesterase B	0	1	0	1
cGMP-inhibited 3',5'-cyclic phosphodiesterase A	0	1	0	1
Cereblon isoform 4	0	2	0	2
vitamin D (1,25- dihydroxyvitamin D3) receptor	3	0	0	3
Insulin-like growth factor-binding protein 5	0	0	1	1
Choline O-acetyltransferase	0	1	0	1
Protein cereblon	0	2	0	2
SMAD family member 2	1	0	0	1
SMAD family member 3	1	0	0	1
TDP1 protein	2	0	0	2
Smad3	1	0	0	1
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)	2	0	0	2
progesterone receptor	2	0	0	2
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a	2	0	0	2
thyroid stimulating hormone receptor	3	0	0	3
nuclear receptor subfamily 1, group I, member 2	1	0	0	1
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
Glucocorticoid receptor	0	2	0	2
Glycine receptor subunit alpha-1	0	2	0	2
Glycine receptor subunit beta	0	2	0	2
Glycine receptor subunit alpha-2	0	2	0	2
Glycine receptor subunit alpha-3	0	2	0	2
ATPase family AAA domain-containing protein 5	2	0	0	2
Ataxin-2	2	0	0	2
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	1	0	1
Asc-type amino acid transporter 1	0	1	0	1
Neutral amino acid transporter B(0)	0	1	0	1
Amino acid transporter	0	1	0	1
Chain A, Protein (aspartate Aminotransferase)	0	0	1	1
Chain A, Aspartate Aminotransferase	0	0	1	1
thyroid hormone receptor beta isoform 2	4	0	0	4
Solute carrier family 22 member 6	0	1	0	3
Solute carrier family 22 member 7	0	0	0	1
Chain B, EUKARYOTIC TRANSLATION INITIATION FACTOR 4E	0	0	1	1
activating transcription factor 6	3	0	0	3
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	3	0	3
Olfactory receptor 51E2	0	0	2	2
Sodium- and chloride-dependent glycine transporter 2	0	1	0	1
Gamma-aminobutyric acid receptor subunit pi	2	1	0	3
Gamma-aminobutyric acid receptor subunit beta-1	2	1	0	3
Gamma-aminobutyric acid receptor subunit delta	2	1	0	3
Gamma-aminobutyric acid receptor subunit gamma-2	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-5	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-3	2	1	0	3
Gamma-aminobutyric acid receptor subunit gamma-1	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-2	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-4	2	1	0	3
Gamma-aminobutyric acid receptor subunit gamma-3	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-6	2	1	0	3
Gamma-aminobutyric acid receptor subunit alpha-1	2	1	0	3
Gamma-aminobutyric acid receptor subunit beta-3	2	1	0	3
Gamma-aminobutyric acid receptor subunit beta-2	2	1	0	3
GABA theta subunit	2	1	0	3
Gamma-aminobutyric acid receptor subunit epsilon	2	1	0	3
Chain A, HISTIDINE-BINDING PROTEIN	0	0	1	1
ATAD5 protein, partial	1	0	0	1
cytochrome P450 3A4 isoform 1	2	0	0	2
Carbonic anhydrase 12	0	0	0	1
Plasma kallikrein	0	0	0	1
Carbonic anhydrase 3	0	0	0	1
Histidine-binding periplasmic protein	0	0	1	1
Carbonic anhydrase 6	0	2	0	3
Adenosine receptor A1	0	0	0	1
Adenosine receptor A2a	0	0	0	1
Carbonic anhydrase 5A, mitochondrial	0	3	0	4
Carbonic anhydrase	0	3	0	4
Carbonic anhydrase 7	0	2	0	3
Carbonic anhydrase	0	0	0	1
Glutaminyl-peptide cyclotransferase	0	1	0	1
Carbonic anhydrase 9	0	3	0	4
Carbonic anhydrase	0	0	0	1
Carbonic anhydrase	0	2	0	3
Carbonic anhydrase	0	1	0	2
Carbonic anhydrase	0	0	0	1
Carbonic anhydrase 13	0	0	0	1
Carbonic anhydrase 15	0	1	0	2
Carbonic anhydrase 13	0	2	0	3
Carbonic anhydrase 14	0	0	0	1
Carbonic anhydrase 5B, mitochondrial	0	2	0	3
Carbonic anhydrase	0	2	0	2
Carbonic anhydrase	0	2	0	2
Carbonic anhydrase, alpha family	0	1	0	1
Transient receptor potential cation channel subfamily A member 1	0	0	1	1
v-jun sarcoma virus 17 oncogene homolog (avian)	3	0	0	3
cellular tumor antigen p53 isoform a	1	0	0	1
Integrin beta-3	1	0	0	1
Integrin alpha-IIb	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	1	0	0	1
Glutathione reductase, mitochondrial	0	2	0	2
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
heat shock protein beta-1	2	0	0	2
Solute carrier family 2, facilitated glucose transporter member 9	0	2	2	4
Microtubule-associated protein tau	2	0	0	2
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)	1	0	0	1
nuclear receptor ROR-gamma isoform 1	2	0	0	2
DNA polymerase kappa isoform 1	2	0	0	2
Serine/threonine-protein kinase D3	0	0	1	1
Protein kinase C gamma type	0	1	1	2
Protein kinase C beta type	0	1	1	2
Luciferin 4-monooxygenase	0	0	1	1
Protein kinase C alpha type	0	1	1	2
Beta-1 adrenergic receptor	0	1	0	1
Protein kinase C eta type	0	0	1	1
Protein kinase C iota type	0	0	1	1
Protein kinase C epsilon type	0	1	1	2
Protein kinase C theta type	0	0	1	1
Protein kinase C zeta type	0	0	1	1
Protein kinase C delta type	0	1	1	2
Serine/threonine-protein kinase D1	0	0	1	1
Ras guanyl-releasing protein 3	0	0	1	1
Chain A, Nuclear Receptor ROR-beta	0	1	0	1
Chain A, Beta-lactamase	2	0	0	2
Chain A, ATP-DEPENDENT DNA HELICASE Q1	1	0	0	1
Chain A, Cruzipain	1	0	0	1
hypoxia-inducible factor 1 alpha subunit	1	0	0	1
USP1 protein, partial	2	0	0	2
thioredoxin glutathione reductase	2	0	0	2
regulator of G-protein signaling 4	1	0	0	1
nonstructural protein 1	1	0	0	1
G	1	0	0	1
Parkin	1	0	0	1
bromodomain adjacent to zinc finger domain 2B	1	0	0	1
aryl hydrocarbon receptor	1	0	0	1
thyrotropin-releasing hormone receptor	1	0	0	1
Caspase-7	1	0	0	1
vitamin D3 receptor isoform VDRA	2	0	0	2
nuclear factor erythroid 2-related factor 2 isoform 2	1	0	0	1
caspase-3	1	0	0	1
geminin	2	0	0	2
peripheral myelin protein 22	2	0	0	2
M-phase phosphoprotein 8	1	0	0	1
Voltage-dependent calcium channel gamma-2 subunit	1	0	0	1
Ornithine decarboxylase	0	0	0	1
Interferon beta	1	0	0	1
HLA class I histocompatibility antigen, B alpha chain	1	0	0	1
Amyloid-beta precursor protein	0	1	0	1
Cytochrome P450 1A2	0	0	1	1
Ornithine decarboxylase	0	0	0	1
60 kDa chaperonin	0	1	0	1
Adenosine receptor A3	0	2	0	2
Retinoic acid receptor alpha	0	2	2	7
Cytochrome P450 2D6	0	0	1	1
60 kDa heat shock protein, mitochondrial	0	1	0	1
Retinoic acid receptor beta	0	2	2	7
Retinoic acid receptor alpha	0	1	2	3
Cytochrome P450 2C9	0	0	1	1
Retinoic acid receptor gamma	0	2	2	7
Alpha-1B adrenergic receptor	0	2	0	2
Alpha-1A adrenergic receptor	0	1	0	1
Retinoic acid receptor gamma	0	1	2	3
5-hydroxytryptamine receptor 1A	0	1	0	1
Glutamate receptor 2	1	0	0	1
Retinoic acid receptor RXR-alpha	0	2	2	4
Retinoic acid receptor beta	0	1	2	3
Cellular retinoic acid-binding protein 2	0	0	1	1
Alpha-1D adrenergic receptor	0	1	0	1
Mitogen-activated protein kinase 1	0	1	0	1
Retinoic acid receptor RXR-alpha	0	1	2	3
Retinoic acid receptor RXR-beta	0	2	2	4
Retinoic acid receptor RXR-beta	0	1	2	3
Retinoic acid receptor RXR-gamma	0	1	2	3
Indoleamine 2,3-dioxygenase 1	0	1	0	1
Cytochrome P450 2C19	0	0	1	1
Nuclear receptor ROR-alpha	0	1	0	1
Alpha-synuclein	0	1	0	1
Cellular retinoic acid-binding protein 1	0	1	0	2
5-hydroxytryptamine receptor 2B	0	2	0	2
Alpha-1A adrenergic receptor	0	2	0	2
Retinoic acid receptor RXR-gamma	0	2	2	4
Nuclear receptor ROR-gamma	0	1	1	2
10 kDa heat shock protein, mitochondrial	0	1	0	1
Cellular retinoic acid-binding protein 1	0	0	1	1
Peptidyl-prolyl cis-trans isomerase NIMA-interacting 1	0	2	1	3
Thiosulfate sulfurtransferase	0	1	0	1
60 kDa chaperonin	0	1	0	1
10 kDa chaperonin	0	1	0	1
Indoleamine 2,3-dioxygenase 2	0	1	0	1
Inositol hexakisphosphate kinase 1	1	0	0	1
Nuclear receptor ROR-beta	0	1	0	1
cytochrome P450 2C9, partial	1	0	0	1
caspase 7, apoptosis-related cysteine protease	1	0	0	1
caspase-3	1	0	0	1
Histone H2A.x	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
Solute carrier family 22 member 8	0	1	0	1
Guanine deaminase	0	1	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Synonyms(1)

Research Excerpts

Overview

Treatment

Research

Studies (120)

Protein Targets (418)

Argininosuccinic Aciduria

Synonyms(1)

Research Excerpts

Overview

Treatment

Research

Studies (120)

Drugs Studied (57)

Protein Targets (418)