Acetyl-CoA acetyltransferase 1 (ACAT1)

Acetyl-CoA acetyltransferase 1 (ACAT1) is a mitochondrial enzyme and a member of the Class II thiolase family that catalyzes the reversible condensation of two molecules of acetyl-CoA to form acetoacetyl-CoA, a key reaction in ketogenesis and cholesterol biosynthesis[1][5][6][8]. ACAT1 is widely expressed in human tissues and plays an essential role in mitochondrial beta-oxidation of fatty acids, energy metabolism, and maintaining acetyl-CoA homeostasis[1][7][8]. Genetic defects in ACAT1 cause beta-ketothiolase deficiency, which can lead to recurrent episodes of ketoacidosis and other metabolic derangements. ACAT1 is distinct from Acyl-CoA:cholesterol acyltransferases (also sometimes abbreviated as ACAT but encoded by different genes, such as SOAT1 and SOAT2); the canonical name outlined here refers specifically to mitochondrial acetyl-CoA acetyltransferase 1[1][5][8].

Other names

Acetoacetyl-CoA thiolaseThiolase IAcetyl coenzyme A acetyltransferase 1T2 (Thiolase II)mitochondrial thiolase3-ketothiolaseAcetyl coenzyme A C-acetyltransferaseKATBeta-ketothiolase

Catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA; drugs would likely act as inhibitors or activators of thiolase activity