KEGG   PATHWAY: hsa00250Help
Entry
hsa00250                    Pathway                                

Name
Alanine, aspartate and glutamate metabolism - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
hsa00250  Alanine, aspartate and glutamate metabolism
hsa00250

Ortholog table
Module
hsa_M00027  GABA (gamma-Aminobutyrate) shunt [PATH:hsa00250]
Disease
H00074  Canavan disease
H00164  Carbamoyl phosphate synthetase I deficiency
H00185  Citrullinemia
H00197  Adenylosuccinate lyase deficiency
H00835  Succinic semialdehyde dehydrogenase deficiency
H00923  Congenital systemic glutamine deficiency
H01028  Argininosuccinic aciduria
H01257  GABA-transaminase deficiency
H01386  Asparagine synthetase deficiency
Drug
D07130  Carglumic acid (JAN/USAN/INN)
Other DBs
BSID: 101142
GO: 0006522 0006531 0006536
Organism
Homo sapiens (human) [GN:hsa]
Gene
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]
259307  IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
8528  DDO; D-aspartate oxidase [KO:K00272] [EC:1.4.3.1]
440  ASNS; asparagine synthetase (glutamine-hydrolyzing) [KO:K01953] [EC:6.3.5.4]
56954  NIT2; nitrilase family member 2 [KO:K13566] [EC:3.5.1.3]
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]
189  AGXT; alanine--glyoxylate and serine--pyruvate aminotransferase [KO:K00830] [EC:2.6.1.51 2.6.1.45 2.6.1.44]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.40 2.6.1.44]
445  ASS1; argininosuccinate synthase 1 [KO:K01940] [EC:6.3.4.5]
435  ASL; argininosuccinate lyase [KO:K01755] [EC:4.3.2.1]
122622  ADSSL1; adenylosuccinate synthase like 1 [KO:K01939] [EC:6.3.4.4]
159  ADSS; adenylosuccinate synthase [KO:K01939] [EC:6.3.4.4]
158  ADSL; adenylosuccinate lyase [KO:K01756] [EC:4.3.2.2]
339983  NAT8L; N-acetyltransferase 8 like [KO:K18309] [EC:2.3.1.17]
57494  RIMKLB; ribosomal modification protein rimK like family member B [KO:K18310] [EC:6.3.2.41 6.3.1.17]
284716  RIMKLA; ribosomal modification protein rimK like family member A [KO:K18311] [EC:6.3.2.42 6.3.2.41]
2346  FOLH1; folate hydrolase 1 [KO:K14592] [EC:3.4.17.21]
443  ASPA; aspartoacylase [KO:K01437] [EC:3.5.1.15]
2571  GAD1; glutamate decarboxylase 1 [KO:K01580] [EC:4.1.1.15]
2572  GAD2; glutamate decarboxylase 2 [KO:K01580] [EC:4.1.1.15]
18  ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.22 2.6.1.19]
7915  ALDH5A1; aldehyde dehydrogenase 5 family member A1 [KO:K00139] [EC:1.2.1.24]
2747  GLUD2; glutamate dehydrogenase 2 [KO:K00261] [EC:1.4.1.3]
2746  GLUD1; glutamate dehydrogenase 1 [KO:K00261] [EC:1.4.1.3]
8659  ALDH4A1; aldehyde dehydrogenase 4 family member A1 [KO:K00294] [EC:1.2.1.88]
2752  GLUL; glutamate-ammonia ligase [KO:K01915] [EC:6.3.1.2]
790  CAD; carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase [KO:K11540] [EC:3.5.2.3 2.1.3.2 6.3.5.5]
27165  GLS2; glutaminase 2 [KO:K01425] [EC:3.5.1.2]
2744  GLS; glutaminase [KO:K01425] [EC:3.5.1.2]
1373  CPS1; carbamoyl-phosphate synthase 1 [KO:K01948] [EC:6.3.4.16]
9945  GFPT2; glutamine-fructose-6-phosphate transaminase 2 [KO:K00820] [EC:2.6.1.16]
2673  GFPT1; glutamine--fructose-6-phosphate transaminase 1 [KO:K00820] [EC:2.6.1.16]
5471  PPAT; phosphoribosyl pyrophosphate amidotransferase [KO:K00764] [EC:2.4.2.14]
Compound
C00014  Ammonia
C00022  Pyruvate
C00025  L-Glutamate
C00026  2-Oxoglutarate
C00036  Oxaloacetate
C00041  L-Alanine
C00042  Succinate
C00049  L-Aspartate
C00064  L-Glutamine
C00122  Fumarate
C00152  L-Asparagine
C00158  Citrate
C00169  Carbamoyl phosphate
C00232  Succinate semialdehyde
C00334  4-Aminobutanoate
C00352  D-Glucosamine 6-phosphate
C00402  D-Aspartate
C00438  N-Carbamoyl-L-aspartate
C00940  2-Oxoglutaramate
C01042  N-Acetyl-L-aspartate
C02362  2-Oxosuccinamate
C03090  5-Phosphoribosylamine
C03406  N-(L-Arginino)succinate
C03794  N6-(1,2-Dicarboxyethyl)-AMP
C03912  (S)-1-Pyrroline-5-carboxylate
C12270  N-Acetylaspartylglutamate
C20775  beta-Citryl-L-glutamate
C20776  N-Acetylaspartylglutamylglutamate
Reference
  Authors
Nishizuka Y, Seyama Y, Ikai A, Ishimura Y, Kawaguchi A (eds).
  Title
[Cellular Functions and Metabolic Maps] (In Japanese)
  Journal
Tokyo Kagaku Dojin (1997)
Reference
PMID:9687539
  Authors
Wu G
  Title
Intestinal mucosal amino acid catabolism.
  Journal
J Nutr 128:1249-52 (1998)
DOI:10.1093/jn/128.8.1249
KO pathway
ko00250   

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