Acyl Coenzyme A Synthetase Medium Chain Family, Member 1 (ACSM1)

ACSM1 has medium-chain fatty acid:CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C₄ to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4-unsaturated acids (in vitro). Functions as GTP-dependent lipoate-activating enzyme that generates the substrate for lipoyltransferase.

The medium chain acyl-CoA dehydrogenase is the best known structure of all acyl-CoA dehydrogenases, and is the most commonly deficient enzyme within the class that leads to metabolic disorders in animals. The molecule is a homotetramer with each subunit containing roughly 400 amino acids and one equivalent of FAD. The tetramer is classified as a “dimer of dimers” with an overall diameter of approximately 90 Å.