ONKI3 | Medical Subject Headings

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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

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Hierarchy:	Medical Subject Headings (MeSH) ∟ Enzymes and Coenzymes ∟ Enzymes ∟ Oxidoreductases ∟ Oxidoreductases Acting on Aldehyde or Oxo Group Donors ∟ Ketone Oxidoreductases ∟ 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) 5 coordinate concepts ∟ 2-Oxoisovalerate Dehydrogenase (Acylating) \| ∟ Ketoglutarate Dehydrogenase Complex \| ∟ Pyruvate Dehydrogenase (Lipoamide) \| ∟ Pyruvate Oxidase \| ∟ Pyruvate Synthase X
Broader terms:	Medical Subject Headings (MeSH) ∟ Enzymes and Coenzymes ∟ Enzymes ∟ Oxidoreductases ∟ Oxidoreductases Acting on Aldehyde or Oxo Group Donors ∟ Ketone Oxidoreductases ∟ 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) Ketone Oxidoreductases > Oxidoreductases Acting on Aldehyde or Oxo Group Donors > Oxidoreductases > Enzymes > Enzymes and Coenzymes > Medical Subject Headings (MeSH) X
History note:	Amino Acids, Branched Chain (1979-1981) \| Carboxy-Lyases (1974-1979) \| Keto Acids (1973-1981) \| Ketone Oxidoreductases (1982-2003) \| Multienzyme Complexes (1982-2003)X
historyNote*:	2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003 X
publicMeSHNote*:	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 X
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. X
activeMeSHYear*:	2006 \| 2007X
dateCreated*:	2003-07-09X
dateEstablished*:	2004-01-01X
dateRevised*:	2005-06-30X
recordAuthorizer*:	sjnX
recordMaintainer*:	jmpX
recordOriginator*:	AKEX
Related term:	Maple Syrup Urine Disease X
Type:	Concept X
Coordinate terms:	2-Oxoisovalerate Dehydrogenase (Acylating) \| Ketoglutarate Dehydrogenase Complex \| Pyruvate Dehydrogenase (Lipoamide) \| Pyruvate Oxidase \| Pyruvate Synthase X
URI:	X
Labels and equivalent concepts:	3-metyyli-2-oksobutanoaattidehydrogenaasi (lipoamidi) (fi) 2-oksoisovaleraattidehydrogenaasi (lipoamidi) (fi, replaced) 3-metyl-2-oxobutanoatdehydrogenas (Lipoamid) (sv) Branched-Chain 2-Oxo Acid Dehydrogenase (en, replaced) Branched-Chain Keto Acid Dehydrogenase (en, replaced) Branched-Chain Oxo-Acid Dehydrogenase (en, replaced) Branched Chain Ketoacid Dehydrogenase (en, replaced) Branched Chain Alpha-Keto Acid Decarboxylase (en, replaced) 2-Oxoisovalerate Dehydrogenase (Lipoamide) (en, replaced) Alpha-Keto Acid Dehydrogenase (en, replaced) BCKA Decarboxylase (en, replaced) 2-Oxoisocaproate Dehydrogenase (en, replaced) X
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