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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) MeSH Descriptor Data 2022


MeSH Heading
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Tree Number(s)
D08.811.682.657.350.760
Unique ID
D042942
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D042942
Scope Note
A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
Entry Term(s)
2-Oxoisocaproate Dehydrogenase
2-Oxoisovalerate Dehydrogenase (Lipoamide)
Alpha-Keto Acid Dehydrogenase
BCKA Decarboxylase
Branched Chain Alpha-Keto Acid Decarboxylase
Branched Chain Ketoacid Dehydrogenase
Branched-Chain 2-Oxo Acid Dehydrogenase
Branched-Chain Keto Acid Dehydrogenase
Branched-Chain Oxo-Acid Dehydrogenase
Registry Number
EC 1.2.4.4
Related Numbers
9082-72-8
CAS Type 1 Name
2-oxoisocaproate dehydrogenase
Previous Indexing
Amino Acids, Branched Chain (1979-1981)
Carboxy-Lyases (1974-1979)
Keto Acids (1973-1981)
Ketone Oxidoreductases (1982-2003)
Multienzyme Complexes (1982-2003)
See Also
Maple Syrup Urine Disease
Public MeSH Note
2004; 2-OXOISOVALERATE DEHYDROGENASE (LIPOAMIDE) (now 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE)) was indexed under KETONE OXIDOREDUCTASES & MULTIENZYME COMPLEXES 1982-2003, under AMINO ACIDS, BRANCH-CHAIN 1979-1981, under KETO ACIDS 1973-1981, under CARBOXY-LYASES 1974-1979
History Note
2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
Date Established
2004/01/01
Date of Entry
2003/07/09
Revision Date
2013/07/09
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) Preferred
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