BCKDH [mitochondrial matrix]

Stable Identifier
R-HSA-70019
Type
Complex
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
lipoylated branched-chain alpha-ketoacid dehydrogenase complex
Locations in the PathwayBrowser
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Literature References
PubMed ID Title Journal Year
2188967 Structure-function relationships in dihydrolipoamide acyltransferases.

Reed, LJ, Hackert, ML

J Biol Chem 1990
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

Beaudet, AL, Scriver, CR, Sly, WS, Valle, D

  2001
10745006 Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Hol, WG, Turley, S, Chuang, DT, Chuang, JL, Wynn, RM, Aevarsson, A

Structure Fold Des 2000
Participants
components
Participates
as an input of
as an output of
Inferred To
BCKDH [mitochondrial matrix] (Bos taurus)
BCKDH [mitochondrial matrix] (Caenorhabditis elegans)
BCKDH [mitochondrial matrix] (Canis familiaris)
BCKDH [mitochondrial matrix] (Danio rerio)
BCKDH [mitochondrial matrix] (Dictyostelium discoideum)
BCKDH [mitochondrial matrix] (Drosophila melanogaster)
BCKDH [mitochondrial matrix] (Gallus gallus)
BCKDH [mitochondrial matrix] (Mus musculus)
BCKDH [mitochondrial matrix] (Plasmodium falciparum)
BCKDH [mitochondrial matrix] (Rattus norvegicus)
BCKDH [mitochondrial matrix] (Sus scrofa)
BCKDH [mitochondrial matrix] (Xenopus tropicalis)
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