3-Ketoacyl CoA thiolase (KAT)

3-Ketoacyl-CoA thiolase (KAT, EC 2.3.1.16) is an enzyme that catalyzes the final step of the β-oxidation pathway, which is essential for fatty acid degradation in both prokaryotes and eukaryotes. This enzyme cleaves a two-carbon acetyl-CoA unit from 3-ketoacyl-CoA substrates, thereby shortening fatty acyl chains and enabling further rounds of β-oxidation or directing products to other metabolic pathways. Deficiency or dysfunction of specific KAT isoforms leads to metabolic disorders such as Medium Chain 3-Ketoacyl-CoA Thiolase Deficiency (MCKAT deficiency), characterized by impaired breakdown of certain fats resulting in toxic metabolite accumulation and energy production deficits. Engineered variants of KAT are being developed for synthetic biology applications.

Other names

Thiolase I

Catalyzes the thiolytic cleavage of 3-ketoacyl-CoAs to release acetyl-CoA and a shortened acyl-CoA.