KEGG    Network variation - Citrate cycle and pyruvate metabolism
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ENTRYnt06031
NameCitrate cycle and pyruvate metabolism
CategoryPathway view; Carbohydrate metabolism
Pathwayhsa00020 Citrate cycle (TCA cycle)
hsa00620 Pyruvate metabolism
Modulehsa_M00307 Pyruvate oxidation, pyruvate => acetyl-CoA
hsa_M00010 Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate
hsa_M00011 Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate
DiseaseH00072 Pyruvate dehydrogenase complex deficiency
H01022 Diseases of the tricarboxylic acid cycle
Display drug-target relation   disease type
N01603    Pyruvate+Lipoyl-E2PDHDLATAcCoA+Dihydrolipoy..
    PDH E1-alpha deficiency   PDHA1*
    PDH E1-beta deficiency   PDHB*
    PDH E2 deficiency     DLAT*
N01609    2OG+Lipoyl-E2OGDHDLSTSucCoA+Dihydrolipo..
    KGDHC deficiency   OGDH*
    YOBELN   OGDHL*
N01616    Dihydrolipoyl-E2DLDLipoyl-E2
    DLD deficiency   DLD*
N01604    OA+AcCoA(CS,ACLY)ACO1/2IDH2OG
    ICRD     ACO2*
    D-2-HGA       IDH2*
N01617    SucCoASUCLSDHFHMDH1/2OA
    SDH deficiency     SDHA*
    SDH deficiency     SDHB*
    SDH deficiency     SDHD*
    FH deficiency       FH*
N01606    PEP(PKLR,PKM)Pyruvate
    PK deficiency   PKLR*
N01605    PyruvatePCOAMDH1/2PCK1/2PEP
    PC deficiency   PC*
    PCKD         PCK1*

Disease nameDisease category
PDH E1-alpha deficiencyH01997Pyruvate dehydrogenase E1-alpha deficiencyInherited metabolic disorder
PDH E1-beta deficiencyH01998Pyruvate dehydrogenase E1-beta deficiencyInherited metabolic disorder
PDH E2 deficiencyH01999Pyruvate dehydrogenase E2 deficiencyInherited metabolic disorder
KGDHC deficiencyH02006Alpha-ketoglutarate dehydrogenase complex deficiencyInherited metabolic disorder, Mitochondrial disease
YOBELNH02562Yoon-Bellen neurodevelopmental syndromeInherited metabolic disorder, Mitochondrial disease
DLD deficiencyH02000Dihydrolipoamide dehydrogenase deficiencyInherited metabolic disorder
ICRDH02113Infantile cerebellar-retinal degenerationInherited metabolic disorder
D-2-HGAH01225D-2-hydroxyglutaric aciduriaInherited metabolic disorder
SDH deficiencyH02005Mitochondrial complex II deficiencyInherited metabolic disorder, Mitochondrial disease
FH deficiencyH02004Fumarase deficiencyInherited metabolic disorder
PK deficiencyH01096Pyruvate kinase deficiencyInherited metabolic disorder
PC deficiencyH00073Pyruvate carboxylase deficiencyInherited metabolic disorder
PCKDH02520Phosphoenolpyruvate carboxykinase deficiencyInherited metabolic disorder