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.

Synonym(s): 3-ketoacyl-CoA thiolase, β-ketothiolase

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.

Home at last, it is the best present of all

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).

Newborn screening for metabolic disorders

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).

Expanded newborn screening for genetic and metabolic disorders: modeling costs and outcomes