Mouse Acetyl Coenzyme A Acetyltransferase 1 (ACAT1) Protein

Background

ACAT1, also known as mitochondrial acetoacetyl-CoA thiolase, is an enzyme localized in the mitochondria that catalyzes the reversible conversion of two molecules of acetyl-CoA into acetoacetyl-CoA, a critical step in ketone body synthesis and cholesterol metabolism. It is involved in energy production during periods of fasting or carbohydrate restriction by contributing to ketogenesis. ACAT1 also plays a role in the breakdown of branched-chain amino acids, linking it to amino acid metabolism. Dysregulation or mutations in the ACAT1 gene have been implicated in rare metabolic disorders such as beta-ketothiolase deficiency, which is characterized by the inability to properly metabolize ketogenic amino acids and fatty acids, leading to metabolic acidosis. Recent studies have also suggested a potential role for ACAT1 in cancer metabolism, where its activity is linked to metabolic reprogramming in tumor cells.