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3-methyl-2-oxobutanoate dehydrogenase (lipoamide) complex

Known as: BCADH, branched-chain alpha-ketoacid dehydrogenase complex, 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) complex location 
A protein complex that catalyzes the reaction 3-methyl-2-oxobutanoate + lipoamide = S-(2-methylpropanoyl)-dihydrolipoamide + carbon dioxide (CO2… 
National Institutes of Health

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Related topics

2 relations
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Broader (1)

Mitochondrial matrix

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2005
Review
2005
Overview of the molecular and biochemical basis of branched-chain amino acid catabolism.
The branched-chain amino acids (BCAAs) are required for protein synthesis and neurotransmitter synthesis. The branched-chain… 
2004
2004
Structural and Biochemical Basis for Novel Mutations in Homozygous Israeli Maple Syrup Urine Disease Patients
Maple syrup urine disease (MSUD) results from mutations affecting different subunits of the mitochondrial branched-chain… 
1998
1998
Impaired Assembly of E1 Decarboxylase of the Branched-chain α-Ketoacid Dehydrogenase Complex in Type IA Maple Syrup Urine Disease*
The E1 decarboxylase component of the human branched-chain ketoacid dehydrogenase complex comprises two E1α (45.5 kDa) and two E1… 
Highly Cited
1995
Highly Cited
1995
A new family of protein kinases--the mitochondrial protein kinases.
Highly Cited
1992
Highly Cited
1992
Branched-chain alpha-ketoacid dehydrogenase kinase. Molecular cloning, expression, and sequence similarity with histidine protein kinases.
Highly Cited
1990
Highly Cited
1990
Primary biliary cirrhosis. Inhibition of pyruvate dehydrogenase complex activity by autoantibodies specific for E1 alpha, a non-lipoic acid containing mitochondrial enzyme.
Antimitochondrial antibodies (AMA) are serologically characteristic of patients with PBC. Four Ag recognized by AMA have been… 
Highly Cited
1990
Highly Cited
1990
Purification and partial characterization of branched-chain alpha-ketoacid dehydrogenase kinase from rat liver and rat heart.
Highly Cited
1990
Highly Cited
1990
Regulation of the branched-chain alpha-ketoacid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease.
Review
1989
Review
1989
Dihydrolipoamide dehydrogenase: functional similarities and divergent evolution of the pyridine nucleotide-disulfide oxidoreductases.
1984
1984
Clofibric acid, phenylpyruvate, and dichloroacetate inhibition of branched-chain alpha-ketoacid dehydrogenase kinase in vitro and in perfused rat heart.
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