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National Drug File - Reference Terminology
Last uploaded:
July 6, 2018
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| Preferred Name | 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) [Chemical/Ingredient] | |
| Synonyms |
Alpha-Keto Acid Dehydrogenase Branched-Chain 2-Oxo Acid Dehydrogenase Branched-Chain Keto Acid Dehydrogenase 2-oxoisocaproate dehydrogenase 2-Oxoisocaproate Dehydrogenase Branched Chain Ketoacid Dehydrogenase Branched-Chain Oxo-Acid Dehydrogenase Branched Chain Alpha-Keto Acid Decarboxylase 2-Oxoisovalerate Dehydrogenase (Lipoamide) 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) BCKA Decarboxylase |
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| ID |
http://purl.bioontology.org/ontology/NDFRT/N0000167916 |
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| altLabel |
Alpha-Keto Acid Dehydrogenase Branched-Chain 2-Oxo Acid Dehydrogenase Branched-Chain Keto Acid Dehydrogenase 2-oxoisocaproate dehydrogenase 2-Oxoisocaproate Dehydrogenase Branched Chain Ketoacid Dehydrogenase Branched-Chain Oxo-Acid Dehydrogenase Branched Chain Alpha-Keto Acid Decarboxylase 2-Oxoisovalerate Dehydrogenase (Lipoamide) 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) BCKA Decarboxylase
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| cui |
C0046494
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| MESH 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.
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| MESH DUI |
D042942
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| MeSH name |
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
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| MESH UI |
M0077442
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| NDFRT kind |
INGREDIENT_KIND
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| notation |
N0000167916
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| NUI |
N0000167916
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| prefLabel |
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) [Chemical/Ingredient]
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| tui |
T116 T126
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| subClassOf |
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