Acyl-CoA synthetase short chain family member 1 (ACSS1)

Acyl-CoA synthetase short chain family member 1 (ACSS1) is a mitochondrial enzyme that catalyzes the conversion of acetate and other short-chain fatty acids into acetyl-CoA, a central metabolic intermediate[1][2][5]. Acetyl-CoA produced by ACSS1 is primarily used for energy production and is particularly vital for cellular energy homeostasis during ketogenic conditions, such as fasting or carbohydrate deprivation[1]. While acetate is its preferred substrate, the enzyme can also utilize propionate with much lower affinity. ACSS1 is involved in metabolic pathways like the TCA cycle and plays a role in thermogenesis. Diseases associated with mutations in ACSS1 include Cystoisosporiasis and Canavan disease[1][5]. The gene is located in the mitochondrial matrix and alternative splice variants exist[2][5].

Other names

Acetyl-coenzyme A synthetase 2-like, mitochondrialACSS1ACAS2LKIAA1846AceCS2dJ568C11.3AceCS2LMGC33843Acetate--CoA ligase 2Acetyl-CoA synthetase 2Propionate--CoA ligaseACECS1