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 |
|---|
| 2002 | 0 | 1 | 1 |
| 2016 | 0 | 1 | 1 |
| 2020 | 1 | 0 | 1 |
| 2025 | 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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Wang J, Poskitt LE, Gallagher J, Puffenberger EG, Wynn RM, Shishodia G, Chuang DT, Beever J, Hardin DL, Brigatti KW, Baker WC, Gately R, Bertrand S, Rodrigues A, Benatti HR, Taghian T, Hall E, Prestigiacomo R, Liang J, Chen G, Zhou X, Ren L, Liu N, He R, Su Q, Xie J, Jiang Z, Gruntman A, Gray-Edwards H, Gao G, Strauss KA, Wang D. BCKDHA-BCKDHB digenic gene therapy restores metabolic homeostasis in two mouse models and a calf with classic maple syrup urine disease. Sci Transl Med. 2025 Feb 26; 17(787):eads0539.
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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.
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Feier F, Schwartz IV, Benkert AR, Seda Neto J, Miura I, Chapchap P, da Fonseca EA, Vieira S, Zanotelli ML, Pinto e Vairo F, Camelo JS, Margutti AV, Mazariegos GV, Puffenberger EG, Strauss KA. Living related versus deceased donor liver transplantation for maple syrup urine disease. Mol Genet Metab. 2016 Mar; 117(3):336-43.
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Morton DH, Strauss KA, Robinson DL, Puffenberger EG, Kelley RI. Diagnosis and treatment of maple syrup disease: a study of 36 patients. Pediatrics. 2002 Jun; 109(6):999-1008.
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Lapointe DS, Hildebrandt E, Buxton DB, Patel TB, Waymack PP, Olson MS. Measurement of branched-chain alpha-keto acid dehydrogenase flux rates in perfused heart and liver. Methods Enzymol. 1988; 166:484-97.