KEGG   PATHWAY: hsa00220Help
Entry
hsa00220                    Pathway                                

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

Ortholog table
Module
hsa_M00029  Urea cycle [PATH:hsa00220]
hsa_M00844  Arginine biosynthesis, ornithine => arginine [PATH:hsa00220]
Network
nt06010  Urea cycle
  Element
N00593  Urea cycle
N00594  ARG1 deficiency in urea cycle
N00595  ASL deficiency in urea cycle
N00596  ASS deficiency in urea cycle
N00597  OTC deficiency in urea cycle
N00598  CPS1 deficiency in urea cycle
N00599  Obligate allosteric activation of CPS1 by NAG
N00600  NAGS deficiency in urea cycle
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)
Other DBs
GO: 0006525
Organism
Homo sapiens (human) [GN:hsa]
Gene
5009  OTC; ornithine carbamoyltransferase [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]
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
KO pathway
ko00220   

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