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Descriptor English: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Descriptor Spanish: 3-Metil-2-Oxobutanoato Deshidrogenasa (Lipoamida)
Descriptor 3-metil-2-oxobutanoato deshidrogenasa (lipoamida)
Entry term(s) 2-oxoisovalerato-deshidrogenasa (lipoamida)
3-metil-2-oxobutanoato-deshidrogenasa (lipoamida)
Scope note: Cetona oxidorreductasa que cataliza la conversión general de alfa-cetoácidos para formar ACIL-CoA y CO2. La enzima necesita DIFOSFATO DE TIAMINA como cofactor. Los defectos en los genes que codifican subunidades de la enzima producen la ENFERMEDAD DE LA ORINA CON OLOR A JARABE DE ARCE. La enzima fue clasificada previamente como EC 1.2.4.3.
Descriptor Portuguese: 3-Metil-2-Oxobutanoato Desidrogenase (Lipoamida)
Descriptor French: 3-Methyl-2-oxobutanoate dehydrogenase (lipoamide)
Entry term(s): 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(s): D08.811.682.657.350.760
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D042942
Scope note: 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.
Allowable Qualifiers: AD administration & dosage
AE adverse effects
AI antagonists & inhibitors
AN analysis
BI biosynthesis
BL blood
CF cerebrospinal fluid
CH chemistry
CL classification
CS chemical synthesis
DE drug effects
DF deficiency
EC economics
GE genetics
HI history
IM immunology
IP isolation & purification
ME metabolism
PD pharmacology
PH physiology
PK pharmacokinetics
PO poisoning
RE radiation effects
SD supply & distribution
ST standards
TO toxicity
TU therapeutic use
UL ultrastructure
UR urine
Registry Number: EC 1.2.4.4
CAS Type 1 Name: 2-oxoisocaproate dehydrogenase
Previous Indexing: Amino Acids, Branched Chain (1979-1981)
Carboxy-Lyases (1974-1979)
Keto Acids (1973-1981)
Ketone Oxidoreductases (1982-2003)
Multienzyme Complexes (1982-2003)
Public MeSH Note: 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
History Note: 2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
Related: Maple Syrup Urine Disease MeSH
DeCS ID: 38207
Unique ID: D042942
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2004/01/01
Date of Entry: 2003/07/09
Revision Date: 2013/07/09
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) - Preferred
Concept UI M0077442
Scope note 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.
Preferred term 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Entry term(s) 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



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