Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADS)

Uniprot Id

P16219

Target Species

Human

Target Name

ACADS

Target Full Name

Short-chain specific acyl-CoA dehydrogenase, mitochondrial

Target Function

Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, short-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 4 to 6 carbons long primary chains.

Target Involvement

Acyl-CoA dehydrogenase short-chain deficiency (ACADSD)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Acyl-CoA dehydrogenase family

Target Synonyms

ACAD3; ACADS; ACADS_HUMAN; Acyl Coenzyme A dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; short chain; Acyl-Coenzyme A dehydrogenase; short chain; AI196007; Bcd-1; Bcd1; Butyryl CoA dehydrogenase; Butyryl-CoA dehydrogenase; EC 1.3.99.2; mitochondrial; SCAD; Short chain acyl CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase; mitochondrial; Unsaturated acyl CoA reductase

Target Background

This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms.