Argininosuccinic acid disodium (BioDeep_00000001299)
Secondary id: BioDeep_00000176200, BioDeep_00000403026, BioDeep_00001872361
natural product human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite
代谢物信息卡片
(2S)-2-[[N-[(4S)-4-amino-4-carboxybutyl]carbamimidoyl]amino]butanedioic acid
化学式: C10H18N4O6 (290.12262880000003)
中文名称: 精氨基琥珀酸
谱图信息:
最多检出来源 Homo sapiens(blood) 0.05%
Reviewed
Last reviewed on 2024-07-02.
Cite this Page
Argininosuccinic acid disodium. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China.
https://query.biodeep.cn/s/argininosuccinic_acid (retrieved
2024-09-17) (BioDeep RN: BioDeep_00000001299). Licensed
under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).
分子结构信息
SMILES: C(=O)(C[C@H](NC(=N)NCCC[C@@H](C(=O)O)N)C(=O)O)O
InChI: InChI=1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)
描述信息
Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the argininosuccinate lyase enzyme can lead to argininosuccinate lyase deficiency, which is an inborn error of metabolism. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (argininosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema.
Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema. [HMDB]
KEIO_ID A039; [MS2] KO008844
KEIO_ID A039
同义名列表
42 个代谢物同义名
(2S)-2-[[N-[(4S)-4-amino-4-carboxybutyl]carbamimidoyl]amino]butanedioic acid; (2S)-2-{N-[(4S)-4-amino-4-carboxybutyl]carbamimidamido}butanedioic acid; N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-aspartic acid; N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-aspartic acid; N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-aspartate; N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-aspartate; N-[(4-Amino-4-carboxybutyl)amidino]-L-aspartic acid; N-(4-Amino-4-carboxybutyl)amidino-L-aspartic acid; N-[(4-Amino-4-carboxybutyl)amidino]-L-aspartate; 2-(N(ω)-L-arginine)succinic acid; 2-(N(ω)-L-arginino)succinic acid; N(ω)-(L-arginino)succinic acid; 2-(N(Omega)-L-arginino)succinic acid; 2-(N(Omega)-L-arginine)succinic acid; Argininosuccinic acid disodium salt; N(Omega)-(L-arginino)succinic acid; 2-(Nomega-L-arginino)succinic acid; (N(omega)-L-arginino)succinic acid; 2-(N(ω)-L-arginino)succinate; 2-(N(ω)-L-arginine)succinate; 2-(Nw-l-arginino)butanedioic acid; 2-(N(Omega)-L-arginino)succinate; N(ω)-(L-arginino)succinate; 2-(N(Omega)-L-arginine)succinate; N(Omega)-(L-arginino)succinate; 2-(Nomega-L-arginino)succinate; 2-(Nw-l-arginino)butanedioate; N-(L-Arginino) succinic acid; N-(L-Arginino)succinic acid; N-(L-Arginino) succinate; L-Argininosuccinic acid; N-(L-Arginino)succinate; Acid, argininosuccinic; Argininosuccinic acid; L-Arginosuccinic acid; L-Argininosuccinate; Arginosuccinic acid; L-Arginosuccinate; ARGININOSUCCINATE; Arginosuccinate; ASA; Argininosuccinic acid
数据库引用编号
39 个数据库交叉引用编号
- ChEBI: CHEBI:184023
- ChEBI: CHEBI:15682
- KEGG: C03406
- PubChem: 16950
- HMDB: HMDB0000052
- Metlin: METLIN389
- Wikipedia: Argininosuccinic acid
- MeSH: Argininosuccinic Acid
- MetaCyc: L-ARGININO-SUCCINATE
- KNApSAcK: C00019688
- foodb: FDB021885
- chemspider: 16059
- CAS: 2387-71-5
- MoNA: KO000073
- MoNA: KO008851
- MoNA: PS017301
- MoNA: KO000074
- MoNA: KO008847
- MoNA: PS017304
- MoNA: KO002110
- MoNA: KO008845
- MoNA: KO002112
- MoNA: PS017302
- MoNA: KO000072
- MoNA: KO000076
- MoNA: KO002108
- MoNA: KO002111
- MoNA: KO008850
- MoNA: KO000075
- MoNA: KO008848
- MoNA: KO008849
- MoNA: PS017303
- MoNA: KO008846
- MoNA: KO008844
- MoNA: KO002109
- PMhub: MS000000284
- 3DMET: B04894
- NIKKAJI: J37.380J
- RefMet: Argininosuccinic acid
分类词条
相关代谢途径
Reactome(0)
PlantCyc(0)
代谢反应
542 个相关的代谢反应过程信息。
Reactome(12)
- Metabolism:
2MACA-CoA + CoA ⟶ Ac-CoA + PROP-CoA - Amino acid and derivative metabolism:
2MACA-CoA + CoA ⟶ Ac-CoA + PROP-CoA - Metabolism of polyamines:
GAA + SAM ⟶ CRET + H+ + SAH - Urea cycle:
CAP + L-Orn ⟶ L-Cit + Pi - Metabolism:
2MACA-CoA + CoA ⟶ Ac-CoA + PROP-CoA - Amino acid and derivative metabolism:
2MACA-CoA + CoA ⟶ Ac-CoA + PROP-CoA - Metabolism of polyamines:
GAA + SAM ⟶ CRET + H+ + SAH - Urea cycle:
CAP + L-Orn ⟶ L-Cit + Pi - Metabolism:
1-3-oxo-THA-CoA + CoA-SH ⟶ DHA-CoA + propionyl CoA - Amino acid and derivative metabolism:
2MACA-CoA + CoA ⟶ Ac-CoA + PROP-CoA - Metabolism of polyamines:
GAA + SAM ⟶ CRET + H+ + SAH - Urea cycle:
ATP + L-Asp + L-Cit ⟶ AMP + ARSUA + PPi
BioCyc(7)
- arginine biosynthesis:
ATP + L-aspartate + citrulline ⟶ AMP + L-arginino-succinate + pyrophosphate - urea cycle:
ATP + L-citrulline + asp ⟶ AMP + H+ + L-arginino-succinate + diphosphate - arginine biosynthesis I:
N-acetyl-L-ornithine + H2O ⟶ L-ornithine + acetate - superpathway of arginine and polyamine biosynthesis:
N-acetyl-L-ornithine + H2O ⟶ L-ornithine + acetate - citrulline-nitric oxide cycle:
L-arginine + NADPH + O2 ⟶ L-citrulline + NADP+ + nitric oxide - arginine biosynthesis I:
N-acetyl-L-ornithine + H2O ⟶ L-ornithine + acetate - arginine biosynthesis II (acetyl cycle):
2-oxoglutarate + N-acetyl-L-ornithine ⟶ N-acetyl-L-glutamate 5-semialdehyde + L-glutamate
Plant Reactome(444)
- Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Amino acid metabolism:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Amino acid metabolism:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Amino acid metabolism:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
L-Glu + imidazole acetol-phosphate ⟶ 2OG + L-histidinol-phosphate - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Amino acid metabolism:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Metabolism and regulation:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Amino acid metabolism:
CoA + NAD + methylmalonate-semialdehyde ⟶ NADH + PROP-CoA + carbon dioxide - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Amino acid biosynthesis:
FAD + PROP-CoA ⟶ FADH2 + acryloyl-CoA - Arginine biosynthesis I:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Arginine biosynthesis II (acetyl cycle):
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi - Citrulline-nitric oxide cycle:
ATP + L-Asp + L-Cit ⟶ AMP + L-Argininosuccinate + PPi
INOH(3)
- Alanine,Aspartic acid and Asparagine metabolism ( Alanine,Aspartic acid and Asparagine metabolism ):
H2O + N-Acetyl-L-aspartic acid ⟶ Acetic acid + L-Aspartic acid - L-Arginino-succinic acid = L-Arginine + Fumaric acid ( Citrate cycle ):
Fumaric acid + L-Arginine ⟶ L-Arginino-succinic acid - Arginine and Proline metabolism ( Arginine and Proline metabolism ):
ATP + Creatine ⟶ ADP + N-Phospho-creatine
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(75)
- Urea Cycle:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Arginine and Proline Metabolism:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolidase Deficiency (PD):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type I:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Ornithine Aminotransferase Deficiency (OAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Canavan Disease:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Hypoacetylaspartia:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Argininemia:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Argininosuccinic Aciduria:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Citrullinemia Type I:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Ornithine Transcarbamylase Deficiency (OTC Deficiency):
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Carbamoyl Phosphate Synthetase Deficiency:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia with Gyrate Atrophy (HOGA):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - L-Arginine:Glycine Amidinotransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Aspartate Metabolism:
Adenosine triphosphate + Ammonia + L-Aspartic acid ⟶ Adenosine monophosphate + L-Asparagine + Pyrophosphate - Arginine Metabolism:
N-Acetylornithine + Water ⟶ Acetic acid + Ornithine - Arginine Metabolism:
N-Acetylornithine + Water ⟶ Acetic acid + Ornithine - Proline Metabolism:
N-Acetylornithine + Water ⟶ Acetic acid + Ornithine - Arginine and Proline Metabolism:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Arginine and Proline Metabolism:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Urea Cycle:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Argininemia:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Argininosuccinic Aciduria:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Canavan Disease:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Carbamoyl Phosphate Synthetase Deficiency:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Citrullinemia Type I:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type I:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hypoacetylaspartia:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Ornithine Aminotransferase Deficiency (OAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolidase Deficiency (PD):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Ornithine Transcarbamylase Deficiency (OTC Deficiency):
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia with Gyrate Atrophy (HOGA):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - L-Arginine:Glycine Amidinotransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Arginine and Proline Metabolism:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Urea Cycle:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Arginine and Proline Metabolism:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Urea Cycle:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Aspartate Metabolism:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Argininemia:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Argininosuccinic Aciduria:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Canavan Disease:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Carbamoyl Phosphate Synthetase Deficiency:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Citrullinemia Type I:
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type I:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperprolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hypoacetylaspartia:
N-Acetyl-L-aspartic acid + Water ⟶ Acetic acid + L-Aspartic acid - Ornithine Aminotransferase Deficiency (OAT Deficiency):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolinemia Type II:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Prolidase Deficiency (PD):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Ornithine Transcarbamylase Deficiency (OTC Deficiency):
Adenosine triphosphate + Citrulline + L-Aspartic acid ⟶ Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate - Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia with Gyrate Atrophy (HOGA):
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - L-Arginine:Glycine Amidinotransferase Deficiency:
Guanidoacetic acid + S-Adenosylmethionine ⟶ Creatine + S-Adenosylhomocysteine - Aspartate Metabolism:
Adenosine triphosphate + L-Aspartic acid + L-Glutamine + Water ⟶ Adenosine monophosphate + L-Asparagine + L-Glutamic acid + Pyrophosphate - Arginine Metabolism:
N-Acetylornithine + Water ⟶ Acetic acid + Ornithine
PharmGKB(0)
12 个相关的物种来源信息
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
- 654 Aeromonas veronii: 10.3389/FCIMB.2020.00044
- 7227 Drosophila melanogaster: 10.1038/S41467-019-11933-Z
- 7461 Apis cerana: 10.1371/JOURNAL.PONE.0175573
- 4896 Schizosaccharomyces pombe: 10.1039/C4MB00346B
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
- 654 Aeromonas veronii: 10.3389/FCIMB.2020.00044
- 7227 Drosophila melanogaster: 10.1038/S41467-019-11933-Z
- 7461 Apis cerana: 10.1371/JOURNAL.PONE.0175573
- 4896 Schizosaccharomyces pombe: 10.1039/C4MB00346B
- 9606 Homo sapiens: -
- 569774 金线莲: -
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Maricruz Mamani-Huanca, Ana Gradillas, Ángeles López-Gonzálvez, Coral Barbas. Unraveling the Cyclization of l-Argininosuccinic Acid in Biological Samples: A Study via Mass Spectrometry and NMR Spectroscopy.
Analytical chemistry.
2020 10; 92(19):12891-12899. doi:
10.1021/acs.analchem.0c01420. [PMID: 32822159] - Bianca Seminotti, Janaína Camacho da Silva, Rafael Teixeira Ribeiro, Guilhian Leipnitz, Moacir Wajner. Free Radical Scavengers Prevent Argininosuccinic Acid-Induced Oxidative Stress in the Brain of Developing Rats: a New Adjuvant Therapy for Argininosuccinate Lyase Deficiency?.
Molecular neurobiology.
2020 Feb; 57(2):1233-1244. doi:
10.1007/s12035-019-01825-0. [PMID: 31707633] - Chandler Griffin, Zineb Ammous, Gail H Vance, Brett H Graham, Marcus J Miller. Rapid quantification of underivatized alloisoleucine and argininosuccinate using mixed-mode chromatography with tandem mass spectrometry.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2019 Oct; 1128(?):121786. doi:
10.1016/j.jchromb.2019.121786. [PMID: 31518899] - Hanna-Riikka Heinonen, Miika Mehine, Netta Mäkinen, Annukka Pasanen, Esa Pitkänen, Auli Karhu, Nanna S Sarvilinna, Jari Sjöberg, Oskari Heikinheimo, Ralf Bützow, Lauri A Aaltonen, Eevi Kaasinen. Global metabolomic profiling of uterine leiomyomas.
British journal of cancer.
2017 Dec; 117(12):1855-1864. doi:
10.1038/bjc.2017.361. [PMID: 29073636] - Julien Baruteau, Elisabeth Jameson, Andrew A Morris, Anupam Chakrapani, Saikat Santra, Suresh Vijay, Huriye Kocadag, Clare E Beesley, Stephanie Grunewald, Elaine Murphy, Maureen Cleary, Helen Mundy, Lara Abulhoul, Alexander Broomfield, Robin Lachmann, Yusof Rahman, Peter H Robinson, Lesley MacPherson, Katharine Foster, W Kling Chong, Deborah A Ridout, Kirsten McKay Bounford, Simon N Waddington, Philippa B Mills, Paul Gissen, James E Davison. Expanding the phenotype in argininosuccinic aciduria: need for new therapies.
Journal of inherited metabolic disease.
2017 05; 40(3):357-368. doi:
10.1007/s10545-017-0022-x. [PMID: 28251416] - Julie Adam, Ming Yang, Christina Bauerschmidt, Mitsuhiro Kitagawa, Linda O'Flaherty, Pratheesh Maheswaran, Gizem Özkan, Natasha Sahgal, Dilair Baban, Keiko Kato, Kaori Saito, Keiko Iino, Kaori Igarashi, Michael Stratford, Christopher Pugh, Daniel A Tennant, Christian Ludwig, Benjamin Davies, Peter J Ratcliffe, Mona El-Bahrawy, Houman Ashrafian, Tomoyoshi Soga, Patrick J Pollard. A role for cytosolic fumarate hydratase in urea cycle metabolism and renal neoplasia.
Cell reports.
2013 May; 3(5):1440-8. doi:
10.1016/j.celrep.2013.04.006. [PMID: 23643539] - Sandesh C S Nagamani, Oleg A Shchelochkov, Mary A Mullins, Susan Carter, Brendan C Lanpher, Qin Sun, Soledad Kleppe, Ayelet Erez, E O'Brian Smith, Juan C Marini, Brendan Lee. A randomized controlled trial to evaluate the effects of high-dose versus low-dose of arginine therapy on hepatic function tests in argininosuccinic aciduria.
Molecular genetics and metabolism.
2012 Nov; 107(3):315-21. doi:
10.1016/j.ymgme.2012.09.016. [PMID: 23040521] - Sandesh C S Nagamani, Ayelet Erez, Brendan Lee. Argininosuccinate lyase deficiency.
Genetics in medicine : official journal of the American College of Medical Genetics.
2012 May; 14(5):501-7. doi:
10.1038/gim.2011.1. [PMID: 22241104] - Dae Eun Choi, Kang Wook Lee, Young Tai Shin, Ki Ryang Na. Hyperammonemia in a patient with late-onset ornithine carbamoyltransferase deficiency.
Journal of Korean medical science.
2012 May; 27(5):556-9. doi:
10.3346/jkms.2012.27.5.556. [PMID: 22563224] - Hyo Jeong Kim, Se Jin Park, Kook In Park, Jin Sung Lee, Ho Sun Eun, Ji Hong Kim, Jae Il Shin. Acute treatment of hyperammonemia by continuous renal replacement therapy in a newborn patient with ornithine transcarbamylase deficiency.
Korean journal of pediatrics.
2011 Oct; 54(10):425-8. doi:
10.3345/kjp.2011.54.10.425. [PMID: 22232626] - Ayelet Erez, Sandesh C Sreenath Nagamani, Brendan Lee. Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond.
American journal of medical genetics. Part C, Seminars in medical genetics.
2011 Feb; 157C(1):45-53. doi:
10.1002/ajmg.c.30289. [PMID: 21312326] - Bee Chin Chen, Lock Hock Ngu, Md Yunus Zabedah. Argininosuccinic aciduria: clinical and biochemical phenotype findings in Malaysian children.
The Malaysian journal of pathology.
2010 Dec; 32(2):87-95. doi:
NULL. [PMID: 21329179] - Susan C Connor, Michael K Hansen, Adam Corner, Randall F Smith, Terence E Ryan. Integration of metabolomics and transcriptomics data to aid biomarker discovery in type 2 diabetes.
Molecular bioSystems.
2010 May; 6(5):909-21. doi:
10.1039/b914182k. [PMID: 20567778] - Ohad Yogev, Orli Yogev, Esti Singer, Eitan Shaulian, Michal Goldberg, Thomas D Fox, Ophry Pines. Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response.
PLoS biology.
2010 Mar; 8(3):e1000328. doi:
10.1371/journal.pbio.1000328. [PMID: 20231875] - Michael Marble, Robin R McGoey, Elizabeth Mannick, Bronya Keats, San San Ng, Stephen Deputy, Heidi Gereighty, Eberhard Schmidt-Sommerfeld. Living related liver transplant in a patient with argininosuccinic aciduria and cirrhosis: metabolic follow-up.
Journal of pediatric gastroenterology and nutrition.
2008 Apr; 46(4):453-6. doi:
10.1097/mpg.0b013e3180ca8720. [PMID: 18367960] - Tanya Newnham, Winita Hardikar, Katrina Allen, R Mark Wellard, Charles Hamilton, Peter Angus, Robert Jones, Avihu Boneh. Liver transplantation for argininosuccinic aciduria: clinical, biochemical, and metabolic outcome.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society.
2008 Jan; 14(1):41-5. doi:
10.1002/lt.21297. [PMID: 18161830] - Narasimhamurthy Shanaiah, M Aruni Desilva, G A Nagana Gowda, Michael A Raftery, Bryan E Hainline, Daniel Raftery. Class selection of amino acid metabolites in body fluids using chemical derivatization and their enhanced 13C NMR.
Proceedings of the National Academy of Sciences of the United States of America.
2007 Jul; 104(28):11540-4. doi:
10.1073/pnas.0704449104. [PMID: 17606902] - Burton D Cohen. Premature aging in uremia.
Molecular and cellular biochemistry.
2007 Apr; 298(1-2):195-8. doi:
10.1007/s11010-006-9351-z. [PMID: 17136440] - David Dimmock, Keiko Kobayashi, Mikio Iijima, Ayako Tabata, Lee-Jun Wong, Takeyori Saheki, Brendan Lee, Fernando Scaglia. Citrin deficiency: a novel cause of failure to thrive that responds to a high-protein, low-carbohydrate diet.
Pediatrics.
2007 Mar; 119(3):e773-7. doi:
10.1542/peds.2006-1950. [PMID: 17332192] - Jill C Fichtel, Judith A Richards, Loretta S Davis. Trichorrhexis nodosa secondary to argininosuccinicaciduria.
Pediatric dermatology.
2007 Jan; 24(1):25-7. doi:
10.1111/j.1525-1470.2007.00327.x. [PMID: 17300644] - Joshua L Deignan, Justin C Livesay, Paul K Yoo, Stephen I Goodman, William E O'Brien, Ramaswamy K Iyer, Stephen D Cederbaum, Wayne W Grody. Ornithine deficiency in the arginase double knockout mouse.
Molecular genetics and metabolism.
2006 Sep; 89(1-2):87-96. doi:
10.1016/j.ymgme.2006.04.007. [PMID: 16753325] - Xavier Stéphenne, Mustapha Najimi, Catherine Sibille, Marie-Cécile Nassogne, Françoise Smets, Etienne M Sokal. Sustained engraftment and tissue enzyme activity after liver cell transplantation for argininosuccinate lyase deficiency.
Gastroenterology.
2006 Apr; 130(4):1317-23. doi:
10.1053/j.gastro.2006.01.008. [PMID: 16618422] - Wim J Kleijer, Victor H Garritsen, Marianne L T van der Sterre, Christoph Berning, Johannes Häberle, Jan G M Huijmans. Prenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia.
Prenatal diagnosis.
2006 Mar; 26(3):242-7. doi:
10.1002/pd.1390. [PMID: 16475226] - Elisabeth L Schwarz, William L Roberts, Marzia Pasquali. Analysis of plasma amino acids by HPLC with photodiode array and fluorescence detection.
Clinica chimica acta; international journal of clinical chemistry.
2005 Apr; 354(1-2):83-90. doi:
10.1016/j.cccn.2004.11.016. [PMID: 15748603] - Claude Bachmann. Long-term outcome of patients with urea cycle disorders and the question of neonatal screening.
European journal of pediatrics.
2003 Dec; 162 Suppl 1(?):S29-33. doi:
10.1007/s00431-003-1347-z. [PMID: 14634803] - Wing Lok Au, Tchoyoson C C Lim, Dennis C C Seow, Pei Lin Koh, Ngai Kun Loh, Maria S F Lim, It Koon Tan, Woon Chee Yee. Serial diffusion-weighted magnetic resonance imaging in adult-onset citrullinaemia.
Journal of the neurological sciences.
2003 May; 209(1-2):101-4. doi:
10.1016/s0022-510x(02)00439-2. [PMID: 12686410] - Burton D Cohen. Methyl group deficiency and guanidino production in uremia.
Molecular and cellular biochemistry.
2003 Feb; 244(1-2):31-6. doi:
. [PMID: 12701806] - Kazumasa Aoyagi. Inhibition of arginine synthesis by urea: a mechanism for arginine deficiency in renal failure which leads to increased hydroxyl radical generation.
Molecular and cellular biochemistry.
2003 Feb; 244(1-2):11-5. doi:
NULL. [PMID: 12701804] - V Reid Sutton, Yanzhen Pan, Erica C Davis, William J Craigen. A mouse model of argininosuccinic aciduria: biochemical characterization.
Molecular genetics and metabolism.
2003 Jan; 78(1):11-6. doi:
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