KEGG   PATHWAY: hsa00220
Entry
hsa00220                    Pathway                                

Name
Arginine biosynthesis - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
Pathway map
hsa00220  Arginine biosynthesis
hsa00220

Module
hsa_M00029  Urea cycle [PATH:hsa00220]
hsa_M00844  Arginine biosynthesis, ornithine => arginine [PATH:hsa00220]
Network
nt06010  Urea cycle
  Element
N00593  Urea cycle
N00599  Obligate allosteric activation of CPS1 by NAG
Disease
H00164  Carbamoyl phosphate synthetase I deficiency
H00185  Citrullinemia
H00186  Hyperargininemia
H00187  Ornithine transcarbamylase deficiency
H00923  Congenital systemic glutamine deficiency
H01028  Argininosuccinic aciduria
H01032  N-acetylglutamate synthase deficiency
H01146  Aminoacylase 1 deficiency
H01398  Primary hyperammonemic disorders (Urea cycle disorders)
Drug
D07130  Carglumic acid (JAN/USAN/INN)
D09018  Tilarginine acetate (USAN)
D11695  Pegzilarginase (USAN/INN)
Other DBs
GO: 0006525
Organism
Homo sapiens (human) [GN:hsa]
Gene
5009  OTC; ornithine transcarbamylase [KO:K00611] [EC:2.1.3.3]
445  ASS1; argininosuccinate synthase 1 [KO:K01940] [EC:6.3.4.5]
435  ASL; argininosuccinate lyase [KO:K01755] [EC:4.3.2.1]
384  ARG2; arginase 2 [KO:K01476] [EC:3.5.3.1]
383  ARG1; arginase 1 [KO:K01476] [EC:3.5.3.1]
4842  NOS1; nitric oxide synthase 1 [KO:K13240] [EC:1.14.13.39]
4843  NOS2; nitric oxide synthase 2 [KO:K13241] [EC:1.14.13.39]
4846  NOS3; nitric oxide synthase 3 [KO:K13242] [EC:1.14.13.39]
27165  GLS2; glutaminase 2 [KO:K01425] [EC:3.5.1.2]
2744  GLS; glutaminase [KO:K01425] [EC:3.5.1.2]
2752  GLUL; glutamate-ammonia ligase [KO:K01915] [EC:6.3.1.2]
2747  GLUD2; glutamate dehydrogenase 2 [KO:K00261] [EC:1.4.1.3]
2746  GLUD1; glutamate dehydrogenase 1 [KO:K00261] [EC:1.4.1.3]
1373  CPS1; carbamoyl-phosphate synthase 1 [KO:K01948] [EC:6.3.4.16]
2805  GOT1; glutamic-oxaloacetic transaminase 1 [KO:K14454] [EC:2.6.1.1]
137362  GOT1L1; glutamic-oxaloacetic transaminase 1 like 1 [KO:K14454] [EC:2.6.1.1]
2806  GOT2; glutamic-oxaloacetic transaminase 2 [KO:K14455] [EC:2.6.1.1]
84706  GPT2; glutamic--pyruvic transaminase 2 [KO:K00814] [EC:2.6.1.2]
2875  GPT; glutamic--pyruvic transaminase [KO:K00814] [EC:2.6.1.2]
162417  NAGS; N-acetylglutamate synthase [KO:K11067] [EC:2.3.1.1]
95  ACY1; aminoacylase 1 [KO:K14677] [EC:3.5.1.14]
100526760  ABHD14A-ACY1; ABHD14A-ACY1 readthrough [KO:K14677] [EC:3.5.1.14]
Compound
C00011  CO2
C00014  Ammonia
C00025  L-Glutamate
C00026  2-Oxoglutarate
C00049  L-Aspartate
C00062  L-Arginine
C00064  L-Glutamine
C00077  L-Ornithine
C00086  Urea
C00122  Fumarate
C00169  Carbamoyl phosphate
C00327  L-Citrulline
C00437  N-Acetylornithine
C00624  N-Acetyl-L-glutamate
C01010  Urea-1-carboxylate
C01250  N-Acetyl-L-glutamate 5-semialdehyde
C03406  N-(L-Arginino)succinate
C04133  N-Acetyl-L-glutamate 5-phosphate
C15532  N-Acetyl-L-citrulline
C20948  LysW-L-glutamate
C20949  LysW-L-glutamyl 5-phosphate
C20950  LysW-L-glutamate 5-semialdehyde
C20951  LysW-L-ornithine
Related
pathway
hsa00020  Citrate cycle (TCA cycle)
hsa00240  Pyrimidine metabolism
hsa00250  Alanine, aspartate and glutamate metabolism
hsa00330  Arginine and proline metabolism
hsa00472  D-Arginine and D-ornithine metabolism
hsa00910  Nitrogen metabolism
KO pathway
ko00220   
LinkDB

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