 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
"3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D042942
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| MeSH Number(s) |
D08.811.682.657.350.760
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| Concept/Terms |
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)- 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
- 2-Oxoisocaproate Dehydrogenase
- 2 Oxoisocaproate Dehydrogenase
- Dehydrogenase, 2-Oxoisocaproate
- 2-Oxoisovalerate Dehydrogenase (Lipoamide)
- Branched-Chain Keto Acid Dehydrogenase
- Branched Chain Keto Acid Dehydrogenase
- BCKA Decarboxylase
- Decarboxylase, BCKA
- Branched Chain Alpha-Keto Acid Decarboxylase
- Branched Chain Alpha Keto Acid Decarboxylase
- Branched Chain Ketoacid Dehydrogenase
- Branched-Chain 2-Oxo Acid Dehydrogenase
- Branched Chain 2 Oxo Acid Dehydrogenase
- Branched-Chain Oxo-Acid Dehydrogenase
- Branched Chain Oxo Acid Dehydrogenase
- Dehydrogenase, Branched-Chain Oxo-Acid
- Oxo-Acid Dehydrogenase, Branched-Chain
- Alpha-Keto Acid Dehydrogenase
- Acid Dehydrogenase, Alpha-Keto
- Alpha Keto Acid Dehydrogenase
- Dehydrogenase, Alpha-Keto Acid
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Below are MeSH descriptors whose meaning is more general than "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)".
Below are MeSH descriptors whose meaning is more specific than "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)".
This graph shows the total number of publications written about "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" by people in this website by year, and whether "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
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| 2015 | 1 | 0 | 1 |
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Below are the most recent publications written about "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" by people in Profiles.
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Jia F, Cui M, Than MT, Han M. Developmental Defects of Caenorhabditis elegans Lacking Branched-chain a-Ketoacid Dehydrogenase Are Mainly Caused by Monomethyl Branched-chain Fatty Acid Deficiency. J Biol Chem. 2016 Feb 05; 291(6):2967-73.
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Skvorak KJ, Dorko K, Marongiu F, Tahan V, Hansel MC, Gramignoli R, Gibson KM, Strom SC. Placental stem cell correction of murine intermediate maple syrup urine disease. Hepatology. 2013 Mar; 57(3):1017-23.
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Skvorak KJ, Paul HS, Dorko K, Marongiu F, Ellis E, Chace D, Ferguson C, Gibson KM, Homanics GE, Strom SC. Hepatocyte transplantation improves phenotype and extends survival in a murine model of intermediate maple syrup urine disease. Mol Ther. 2009 Jul; 17(7):1266-73.
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