mutant BCKDHA:BCKDHB tetramer [mitochondrial matrix]

Stable Identifier
R-HSA-9865107
Type
Complex
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
2xBCKDHA:2xBCKDHB:2xTDP, branched-chain alpha-ketoacid dehydrogenase E1 complex
Locations in the PathwayBrowser
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Participants
components
Participates
as a member of
Disease
Name Identifier Synonyms
maple syrup urine disease DOID:9269 dihydrolipoamide dehydrogenase deficiency
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