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DeCS
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Descriptor English:
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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
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Descriptor Spanish:
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3-Metil-2-Oxobutanoato Deshidrogenasa (Lipoamida)
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Descriptor Portuguese:
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3-Metil-2-Oxobutanoato Desidrogenase (Lipoamida)
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Synonyms English:
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2-Oxoisocaproate Dehydrogenase
Alpha-Keto Acid Dehydrogenase
BCKA Decarboxylase
Branched Chain Alpha-Keto Acid Decarboxylase
Branched Chain Ketoacid Dehydrogenase
Branched-Chain 2-Oxo Acid Dehydrogenase
Branched-Chain Keto Acid Dehydrogenase
Branched-Chain Oxo-Acid Dehydrogenase
2 Oxoisocaproate Dehydrogenase
Acid Dehydrogenase, Alpha-Keto
Alpha Keto Acid Dehydrogenase
Branched Chain 2 Oxo Acid Dehydrogenase
Branched Chain Alpha Keto Acid Decarboxylase
Branched Chain Keto Acid Dehydrogenase
Branched Chain Oxo Acid Dehydrogenase
Decarboxylase, BCKA
Dehydrogenase, 2-Oxoisocaproate
Dehydrogenase, Alpha-Keto Acid
Dehydrogenase, Branched-Chain Oxo-Acid
Oxo-Acid Dehydrogenase, Branched-Chain
2-Oxoisovalerate Dehydrogenase (Lipoamide)
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Tree Number:
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D08.811.682.657.350.760
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Definition English:
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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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See Related English:
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Maple Syrup Urine Disease
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History Note English:
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2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
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Allowable Qualifiers English:
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Record Number:
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38207
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Unique Identifier:
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D042942
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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