Gene: [06p2/BCKDE1B] 2-oxoisovalerate dehydrogenase (lipoamide), beta polypeptide; branched chain keto acid dehydrogenase E1, beta polypeptide; maple syrup urine disease (due to BCK-E1-beta deficiency);

FAG	The branched-chain ketoacid dehydrogenase alpha subunit gene have been mapped to chromosome 19; see also GEM:19q132/BCKDE1A."
FUN	The holoenzyme functions as a component of multienzyme complex BCKD, which decarboxylates branched chain keto acids, adds them to coenzyme A, and synthesizes NADH."
PAT	See GEM:19q132/BCKDE1A.
REF	CLO,SEQ "Chuang JL &: FEBS Lett, 262, 305-309, 1990 PAT,PHE,MEB "Indo Y &: J Clin Invest, 80, N1, 63-70, 1987 LOC,FAG "Zneimer SM &: Genomics, 10, N3, 740-747, 1991
KEY	neu, mtbd, aac, lip, mito
CLA	coding, basic
LOC	06 p22-21
MIM	MIM: 248611
EZN	ENZYME: 1.2.4.4