acetyl-CoA acyltransferase

(redirected from beta-ketothiolase)

a·ce·tyl-CoA ac·yl·trans·fer·ase

an enzyme catalyzing the thioclastic cleavage by coenzyme A of β-ketoacyl-CoA, forming an acyl-CoA with a carbon chain shorter by two atoms, the missing two atoms appearing as acetyl-CoA. A step in fatty acid degradation.
See also: acetyl-CoA acetyltransferase.
References in periodicals archive ?
The nearest there has been to a diagnosis was when it was suggested he could have beta-ketothiolase deficiency, a rare condition which impairs the body's ability to breakdown fat.
The organic acid disorders tested in newborn screening include methylmalonic acidemia (MMA), propionic acidemia (PA), isovaleric acidemia (IVA), 3-methylcrotonyl-coenzyme A carboxylase (3-MCC) deficiency, multiple carboxylase deficiency (MCD), glutaric aciduria type 1 and 2 (GA-1 and GA-2), beta-ketothiolase (BKT) deficiency, and 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency (HMG-CoA).
Based on specific evaluation criteria, the disorders were ranked from 1 (highest priority) to 29, with Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) being first in the ranking and Beta-ketothiolase (BKT) ranking number 29 (ACMG, 2005).