KEGG    Network variation - Valine, leucine and isoleucine degradation
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ENTRYnt06024
NameValine, leucine and isoleucine degradation
CategoryPathway view; Amino acid metabolism
Pathwayhsa00280 Valine, leucine and isoleucine degradation
Modulehsa_M00036 Leucine degradation, leucine => acetoacetate + acetyl-CoA
hsa_M00741 Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA
Display drug-target relation   disease type
N00832    [Leu,Val,Ile]−BCAT1/2→[KIC,KIV,KMV]
N01619    [KIC,KIV,KMV]+Lipo..−BCKDH⇉DBT→[IV-CoA,IB-CoA,2-M..
    MSUD (type Ia)   BCKDHA*
    MSUD (type Ib)   BCKDHB*
    MSUD (type II)     DBT*
N01616    Dihydrolipoyl-E2−DLD→Lipoyl-E2
    MSUD (type III)   DLD*
N00851    IV-CoA−(IVD,ACADM)⇉MCCC1/2⇉AUH⇉(HMGCL,HMGCLL1)→AcCoA
    Isovaleric acidemia   IVD*
    3-Methylcrotonylglycinuria     MCCC1*
    3-Methylcrotonylglycinuria     MCCC2*
    MGCA1       AUH*
    HL deficiency         HMGCL*
N00852    IB-CoA−(ACADS,ACAD8,ACADM..⇉(ECHS1,HADHA)⇉HIBCH⇉HIBADH⇉ALDH6A1→Propanoyl-CoA
    IBD deficiency   ACAD8*
    Methacrylic aciduria       HIBCH*
    MMSDH deficiency           ALDH6A1*
N00856    2-MB-CoA−(ACADS,ACADSB,ACAD..⇉(ECHS1,HADHA)⇉(HSD17B10,HADH)⇉(ACAA1/2,HADHB)→Propanoyl-CoA
    SBCAD deficiency   ACADSB*
    MHBD deficiency       HSD17B10*
N00842    Propanoyl-CoA−(PCCA+PCCB)⇉MCEE⇉MMUT→Succinyl-CoA
    Propionic acidemia   PCCA*
    Propionic acidemia   PCCB*
    MCE deficiency     MCEE*
    MMA mut type       MMUT*

Disease nameDisease category
MSUD (type Ia)H00172Maple syrup urine diseaseInherited metabolic disorder
MSUD (type Ib)H00172Maple syrup urine diseaseInherited metabolic disorder
MSUD (type II)H00172Maple syrup urine diseaseInherited metabolic disorder
MSUD (type III)H02000Dihydrolipoamide dehydrogenase deficiencyInherited metabolic disorder
Isovaleric acidemiaH00173Isovaleric acidemiaInherited metabolic disorder
3-MethylcrotonylglycinuriaH001813-MethylcrotonylglycinuriaInherited metabolic disorder
MGCA1H007543-Methylglutaconic aciduriaInherited metabolic disorder
HL deficiencyH001793-Hydroxy-3-methylglutaryl-CoA lyase deficiencyInherited metabolic disorder
IBD deficiencyH01279Isobutyryl-CoA dehydrogenase deficiencyInherited metabolic disorder
Methacrylic aciduriaH01349Methacrylic aciduriaInherited metabolic disorder, Mitochondrial disease
MMSDH deficiencyH02285Methylmalonate semialdehyde dehydrogenase deficiencyInherited metabolic disorder
SBCAD deficiencyH00375SBCAD deficiencyInherited metabolic disorder
MHBD deficiencyH009252-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiencyInherited metabolic disorder
Propionic acidemiaH00175Propionic acidemiaInherited metabolic disorder
MCE deficiencyH00174Methylmalonic aciduriaInherited metabolic disorder
MMA mut typeH00174Methylmalonic aciduriaInherited metabolic disorder