KEGG   PATHWAY: hsa00280Help
Entry
hsa00280                    Pathway                                

Name
Valine, leucine and isoleucine degradation - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
Valine, leucine and isoleucine degradation
hsa00280

All organismsOrtholog table
Module
Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:hsa00280]
Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:hsa00280]
Disease
H00172  
Maple syrup urine disease (MSUD)
H00173  
Isovaleric acidemia (IVA)
H00174  
Methylmalonic aciduria (MMA)
H00175  
Propionic acidemia
H00179  
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H00181  
3-Methylcrotonylglycinuria
H00525  
Disorders of fatty-acid oxidation
H00754  
3-Methylglutaconic aciduria (MGCA)
H00925  
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency
H01076  
Alpha-methylacetoacetic aciduria
H01121  
Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
H01123  
HMG-CoA synthase (HMGCS) deficiency
H01279  
Isobutyryl-CoA dehydrogenase (IBD) deficiency
H01349  
Methacrylic aciduria
H01364  
3-Hydroxyacyl-CoA dehydrogenase deficiency
H01400  
Secondary hyperammonemia
Drug
D00123  
Cyanamide (JP17)
D00131  
Disulfiram (JP17/USP/INN)
D00399  
Valproic acid (USP)
D00535  
Vigabatrin (JAN/USAN/INN)
D00710  
Valproate sodium (USAN)
D08667  
Calcium valproate
Other DBs
Organism
Homo sapiens (human) [GN:hsa]
Gene
587  
BCAT2; branched chain amino acid transaminase 2 [KO:K00826] [EC:2.6.1.42]
586  
BCAT1; branched chain amino acid transaminase 1 [KO:K00826] [EC:2.6.1.42]
259307  
IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
593  
BCKDHA; branched chain keto acid dehydrogenase E1, alpha polypeptide [KO:K00166] [EC:1.2.4.4]
594  
BCKDHB; branched chain keto acid dehydrogenase E1 subunit beta [KO:K00167] [EC:1.2.4.4]
1629  
DBT; dihydrolipoamide branched chain transacylase E2 [KO:K09699] [EC:2.3.1.168]
1738  
DLD; dihydrolipoamide dehydrogenase [KO:K00382] [EC:1.8.1.4]
35  
ACADS; acyl-CoA dehydrogenase, C-2 to C-3 short chain [KO:K00248] [EC:1.3.8.1]
34  
ACADM; acyl-CoA dehydrogenase, C-4 to C-12 straight chain [KO:K00249] [EC:1.3.8.7]
3712  
IVD; isovaleryl-CoA dehydrogenase [KO:K00253] [EC:1.3.8.4]
36  
ACADSB; acyl-CoA dehydrogenase, short/branched chain [KO:K09478] [EC:1.3.99.12]
27034  
ACAD8; acyl-CoA dehydrogenase family member 8 [KO:K11538] [EC:1.3.99.-]
3030  
HADHA; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  
EHHADH; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]
1892  
ECHS1; enoyl-CoA hydratase, short chain 1 [KO:K07511] [EC:4.2.1.17]
3033  
HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3028  
HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 [KO:K08683] [EC:1.1.1.178 1.1.1.35]
30  
ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
10449  
ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
3032  
HADHB; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit [KO:K07509] [EC:2.3.1.16]
5095  
PCCA; propionyl-CoA carboxylase alpha subunit [KO:K01965] [EC:6.4.1.3]
5096  
PCCB; propionyl-CoA carboxylase beta subunit [KO:K01966] [EC:6.4.1.3]
84693  
MCEE; methylmalonyl-CoA epimerase [KO:K05606] [EC:5.1.99.1]
4594  
MUT; methylmalonyl-CoA mutase [KO:K01847] [EC:5.4.99.2]
26275  
HIBCH; 3-hydroxyisobutyryl-CoA hydrolase [KO:K05605] [EC:3.1.2.4]
11112  
HIBADH; 3-hydroxyisobutyrate dehydrogenase [KO:K00020] [EC:1.1.1.31]
4329  
ALDH6A1; aldehyde dehydrogenase 6 family member A1 [KO:K00140] [EC:1.2.1.27 1.2.1.18]
217  
ALDH2; aldehyde dehydrogenase 2 family (mitochondrial) [KO:K00128] [EC:1.2.1.3]
224  
ALDH3A2; aldehyde dehydrogenase 3 family member A2 [KO:K00128] [EC:1.2.1.3]
219  
ALDH1B1; aldehyde dehydrogenase 1 family member B1 [KO:K00128] [EC:1.2.1.3]
501  
ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.3 1.2.1.8 1.2.1.31]
223  
ALDH9A1; aldehyde dehydrogenase 9 family member A1 [KO:K00149] [EC:1.2.1.3 1.2.1.47]
316  
AOX1; aldehyde oxidase 1 [KO:K00157] [EC:1.2.3.1]
197322  
ACSF3; acyl-CoA synthetase family member 3 [KO:K18660] [EC:6.2.1.-]
18  
ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.22 2.6.1.19]
64902  
AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.40 2.6.1.44]
56922  
MCCC1; methylcrotonoyl-CoA carboxylase 1 [KO:K01968] [EC:6.4.1.4]
64087  
MCCC2; methylcrotonoyl-CoA carboxylase 2 [KO:K01969] [EC:6.4.1.4]
549  
AUH; AU RNA binding protein/enoyl-CoA hydratase [KO:K05607] [EC:4.2.1.18]
3155  
HMGCL; 3-hydroxymethyl-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]
54511  
HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1 [KO:K01640] [EC:4.1.3.4]
5019  
OXCT1; 3-oxoacid CoA-transferase 1 [KO:K01027] [EC:2.8.3.5]
64064  
OXCT2; 3-oxoacid CoA-transferase 2 [KO:K01027] [EC:2.8.3.5]
65985  
AACS; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]
39  
ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
38  
ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
3157  
HMGCS1; 3-hydroxy-3-methylglutaryl-CoA synthase 1 [KO:K01641] [EC:2.3.3.10]
3158  
HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 [KO:K01641] [EC:2.3.3.10]
Compound
C00024  
Acetyl-CoA
C00068  
Thiamin diphosphate
C00091  
Succinyl-CoA
C00100  
Propanoyl-CoA
C00123  
L-Leucine
C00141  
3-Methyl-2-oxobutanoic acid
C00164  
Acetoacetate
C00183  
L-Valine
C00233  
4-Methyl-2-oxopentanoate
C00332  
Acetoacetyl-CoA
C00349  
2-Methyl-3-oxopropanoate
C00356  
(S)-3-Hydroxy-3-methylglutaryl-CoA
C00407  
L-Isoleucine
C00630  
2-Methylpropanoyl-CoA
C00671  
(S)-3-Methyl-2-oxopentanoic acid
C00683  
(S)-Methylmalonyl-CoA
C01205  
(R)-3-Amino-2-methylpropanoate
C01213  
(R)-Methylmalonyl-CoA
C02170  
Methylmalonate
C02939  
3-Methylbutanoyl-CoA
C03069  
3-Methylcrotonyl-CoA
C03231  
3-Methylglutaconyl-CoA
C03284  
L-3-Aminoisobutanoate
C03344  
2-Methylacetoacetyl-CoA
C03345  
2-Methylbut-2-enoyl-CoA
C03460  
2-Methylprop-2-enoyl-CoA
C04405  
(2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA
C05996  
Branched chain fatty acid
C05998  
3-Hydroxyisovaleryl-CoA
C06000  
(S)-3-Hydroxyisobutyryl-CoA
C06001  
(S)-3-Hydroxyisobutyrate
C06002  
(S)-Methylmalonate semialdehyde
C15972  
Enzyme N6-(lipoyl)lysine
C15973  
Enzyme N6-(dihydrolipoyl)lysine
C15974  
3-Methyl-1-hydroxybutyl-ThPP
C15975  
[Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(3-methylbutanoyl)dihydrolipoyllysine
C15976  
2-Methyl-1-hydroxypropyl-ThPP
C15977  
[Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
C15978  
2-Methyl-1-hydroxybutyl-ThPP
C15979  
[Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine
C15980  
(S)-2-Methylbutanoyl-CoA
C20827  
3-Hydroxyisovalerate
KO pathway
 

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