KEGG   PATHWAY: hsa00250Help
Entry
hsa00250                    Pathway                                

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

All organismsOrtholog table
Module
GABA (gamma-Aminobutyrate) shunt [PATH:hsa00250]
Disease
H00074  
Canavan disease (CD)
H00164  
Carbamoyl phosphate synthetase I deficiency
H00185  
Citrullinemia (CTLN)
H00197  
Adenylosuccinate lyase deficiency
H00835  
Succinic semialdehyde dehydrogenase (SSADH) deficiency
H00923  
Congenital systemic glutamine deficiency (CSGD)
H01028  
Argininosuccinic aciduria (ARGINSA)
H01097  
Cerebral palsy
H01257  
GABA-transaminase deficiency
H01386  
Asparagine synthetase deficiency
Drug
D00399  
Valproic acid (USP)
D00535  
Vigabatrin (JAN/USAN/INN)
D00710  
Valproate sodium (USAN)
D07130  
Carglumic acid (JAN/USAN/INN)
D08667  
Calcium valproate
Other DBs
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 pyruvate transaminase (alanine aminotransferase) 2 [KO:K00814] [EC:2.6.1.2]
2875  
GPT; glutamic-pyruvate transaminase (alanine aminotransferase) [KO:K00814] [EC:2.6.1.2]
189  
AGXT; alanine-glyoxylate 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 (prostate-specific membrane antigen) 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)
KO pathway
 

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