Ketonoxidoreduktáza, která katalyzuje celkovou konverzi alfa-ketokyselin na ACYL-CoA a CO2. Enzym vyžaduje thiamindifosfát (THIAMINPYROFOSFÁT) jako kofaktor. Defekty genů, které kódují podjednotky enzymu, jsou příčinou nemoci s močí javorového sirupu. Enzym byl dříve klasifikován jako EC 1.2.4.3.

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.

DUI

D042942 MeSH Prohlížeč

CUI

M0077442

CAS

2-oxoisocaproate dehydrogenase

Předchozí užití

Amino Acids, Branched Chain (1979-1981); Carboxy-Lyases (1974-1979); Keto Acids (1973-1981); Ketone Oxidoreductases (1982-2003); Multienzyme Complexes (1982-2003)

Historická pozn.

2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003

Veřejná pozn.

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