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BCKDHA:BCKDHB tetramer [mitochondrial matrix]
Stable Identifier
R-HSA-70016
Type
Complex
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
2xBCKDHA:2xBCKDHB:2xTDP, branched-chain alpha-ketoacid dehydrogenase E1 complex
Locations in the PathwayBrowser
Expand all
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of branched-chain amino acid catabolism (Homo sapiens)
Branched-chain ketoacid dehydrogenase kinase deficiency (Homo sapiens)
BCKDK loss-of-function mutations do not phosphorylate BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
Maple Syrup Urine Disease (Homo sapiens)
Loss-of-function mutations in DBT cause MSUD2 (Homo sapiens)
DBT loss-of-function mutants don't synthesize BCAA-CoA (Homo sapiens)
DBT mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
Loss-of-function mutations in DLD cause MSUD3/DLDD (Homo sapiens)
Loss-of-function DLD mutants don't dehydrogenate dihydrolipoyl DBT (Homo sapiens)
DLD mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
Metabolism (Homo sapiens)
Metabolism of amino acids and derivatives (Homo sapiens)
Branched-chain amino acid catabolism (Homo sapiens)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Homo sapiens)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
DBT transfers BCAA to CoA (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDK phosphorylates BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
PPM1K dephosphorylates p-BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
Participants
components
x 2
BCKDHA [mitochondrial matrix]
(Homo sapiens)
x 2
BCKDHB [mitochondrial matrix]
(Homo sapiens)
x 4
K+ [mitochondrial matrix]
x 2
TDP [mitochondrial matrix]
Participates
as a component of
DBT mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
DLD mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
Inferred To
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Bos taurus)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Caenorhabditis elegans)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Canis familiaris)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Danio rerio)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Dictyostelium discoideum)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Drosophila melanogaster)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Gallus gallus)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Mus musculus)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Plasmodium falciparum)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Rattus norvegicus)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Sus scrofa)
BCKDHA:BCKDHB tetramer [mitochondrial matrix]
(Xenopus tropicalis)
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