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Medical Subject Headings
Last uploaded:
March 22, 2026
| Id | http://purl.bioontology.org/ontology/MESH/D042942
http://purl.bioontology.org/ontology/MESH/D042942
|
|---|---|
| Preferred Name | 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) |
| Definitions |
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.
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| Synonyms |
Alpha-Keto Acid Dehydrogenase
Acid Dehydrogenase, Alpha-Keto
Oxo-Acid Dehydrogenase, Branched-Chain
Branched-Chain 2-Oxo Acid Dehydrogenase
Dehydrogenase, Branched-Chain Oxo-Acid
Decarboxylase, BCKA
Branched-Chain Keto Acid Dehydrogenase
2-oxoisocaproate dehydrogenase
2-Oxoisocaproate Dehydrogenase
Branched Chain Oxo Acid Dehydrogenase
Branched Chain Ketoacid Dehydrogenase
Dehydrogenase, Alpha-Keto Acid
Branched-Chain Oxo-Acid Dehydrogenase
Branched Chain Keto Acid Dehydrogenase
Branched Chain Alpha-Keto Acid Decarboxylase
2-Oxoisovalerate Dehydrogenase (Lipoamide)
2 Oxoisocaproate Dehydrogenase
Dehydrogenase, 2-Oxoisocaproate
Branched Chain Alpha Keto Acid Decarboxylase
Branched Chain 2 Oxo Acid Dehydrogenase
BCKA Decarboxylase
Alpha Keto Acid Dehydrogenase
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|
| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| definition | 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. |
|---|---|
| altLabel |
Alpha-Keto Acid Dehydrogenase
Acid Dehydrogenase, Alpha-Keto
Oxo-Acid Dehydrogenase, Branched-Chain
Branched-Chain 2-Oxo Acid Dehydrogenase
Dehydrogenase, Branched-Chain Oxo-Acid
Decarboxylase, BCKA
Branched-Chain Keto Acid Dehydrogenase
2-oxoisocaproate dehydrogenase
2-Oxoisocaproate Dehydrogenase
Branched Chain Oxo Acid Dehydrogenase
Branched Chain Ketoacid Dehydrogenase
Dehydrogenase, Alpha-Keto Acid
Branched-Chain Oxo-Acid Dehydrogenase
Branched Chain Keto Acid Dehydrogenase
Branched Chain Alpha-Keto Acid Decarboxylase
2-Oxoisovalerate Dehydrogenase (Lipoamide)
2 Oxoisocaproate Dehydrogenase
Dehydrogenase, 2-Oxoisocaproate
Branched Chain Alpha Keto Acid Decarboxylase
Branched Chain 2 Oxo Acid Dehydrogenase
BCKA Decarboxylase
Alpha Keto Acid Dehydrogenase
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| prefLabel | 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
|
| Machine permutation | 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
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| HN | 2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
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| type | |
| tui |
T116
T126
|
| TERMUI |
T107440
T107441
T107433
T526353
T107432
T107443
T107437
T107445
T107438
T107444
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| TH | NLM (2004)
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| MMR | 20130709
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| notation | D042942
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| Inverse of RO | |
| Semantic type UMLS property | |
| RR | 9082-72-8 (CRN)
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| Inverse of AQ |
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| FX | D008375
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| DX | 20040101
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| cui | C0046494
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| DC | 1
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| MN | D08.811.682.657.350.760
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| AQL | AD AE AI AN BI BL CF CH CL CS DE DF EC GE HI IM IP ME PD PH PK PO RE SD ST TO TU UL UR
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| Inverse of RB | EC 1.2.4.4
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| MDA | 20021118
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| subClassOf |
| Delete | Subject | Author | Type | Created |
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| No notes to display |