Search on: BRANCHED-CHAIN 2-OXO ACID DEHYDROGENASE 
Descriptors Found: 1
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Descriptor English:   3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) 
Descriptor Spanish:   3-Metil-2-Oxobutanoato Deshidrogenasa (Lipoamida) 
Descriptor Portuguese:   3-Metil-2-Oxobutanoato Desidrogenase (Lipoamida) 
Synonyms English:   2 Oxoisocaproate Dehydrogenase
2-Oxoisocaproate Dehydrogenase
2-Oxoisovalerate Dehydrogenase (Lipoamide)
Acid Dehydrogenase, Alpha-Keto
Alpha Keto Acid Dehydrogenase
Alpha-Keto Acid Dehydrogenase
BCKA Decarboxylase
Branched Chain 2 Oxo Acid Dehydrogenase
Branched Chain Alpha Keto Acid Decarboxylase
Branched Chain Alpha-Keto Acid Decarboxylase
Branched Chain Keto Acid Dehydrogenase
Branched Chain Ketoacid Dehydrogenase
Branched Chain Oxo Acid Dehydrogenase
Branched-Chain 2-Oxo Acid Dehydrogenase
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  
Tree Number:   D08.811.682.657.350.760
Definition English:   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. 
See Related English:   Maple Syrup Urine Disease
 
History Note English:   2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003 
Allowable Qualifiers English:  
AD administration & dosage AE adverse effects
AN analysis AI antagonists & inhibitors
BI biosynthesis BL blood
CF cerebrospinal fluid CS chemical synthesis
CH chemistry CL classification
DF deficiency DE drug effects
EC economics GE genetics
HI history IM immunology
IP isolation & purification ME metabolism
PK pharmacokinetics PD pharmacology
PH physiology PO poisoning
RE radiation effects ST standards
SD supply & distribution TU therapeutic use
TO toxicity UL ultrastructure
UR urine  
Record Number:   38207 
Unique Identifier:   D042942 

Occurrence in VHL:
 

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