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
|
| MeSH Number(s) |
D08.811.682.657.350.760
|
| 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 |
|---|
| 2020 | 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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Strauss KA, Carson VJ, Soltys K, Young ME, Bowser LE, Puffenberger EG, Brigatti KW, Williams KB, Robinson DL, Hendrickson C, Beiler K, Taylor CM, Haas-Givler B, Chopko S, Hailey J, Muelly ER, Shellmer DA, Radcliff Z, Rodrigues A, Loeven K, Heaps AD, Mazariegos GV, Morton DH. Branched-chain a-ketoacid dehydrogenase deficiency (maple syrup urine disease): Treatment, biomarkers, and outcomes. Mol Genet Metab. 2020 03; 129(3):193-206.