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

Molecular Classification

Enzyme

Other Names

Acetyl-coenzyme A synthetase 2-like, mitochondrial, ACSS1, ACAS2L, KIAA1846, AceCS2, dJ568C11.3, AceCS2L, MGC33843, Acetate--CoA ligase 2, Acetyl-CoA synthetase 2, Propionate--CoA ligase, ACECS1

Disease Roles

CancerOther (e.g. Cystoisosporiasis, Canavan disease)

Acyl-CoA synthetase short chain family member 1 Overview

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].

Mechanism of Action

Biological Functions

Acetyl-CoA biosynthetic process

Acetyl-CoA synthesis from short-chain fatty acids (mainly acetate)

Tricarboxylic acid (TCA) cycle

Thermogenesis under ketogenic conditions

Disease Associations

Cancer

Other (e.g. Cystoisosporiasis, Canavan disease)

Safety Considerations

No safety concerns listed