The ACOX1 gene provides instructions for making an enzyme called peroxisomal straight-chain acyl-CoA oxidase. This enzyme is found in sac-like cell structures (organelles) called peroxisomes, which contain a variety of enzymes that break down many different substances. The peroxisomal straight-chain acyl-CoA oxidase enzyme plays a role in the breakdown of certain fat molecules called very long-chain fatty acids (VLCFAs). Specifically, it is involved in the first step of a process called the peroxisomal fatty acid beta-oxidation pathway. This process shortens the VLCFA molecules by two carbon atoms at a time until the VLCFAs are converted to a molecule called acetyl-CoA, which is transported out of the peroxisomes for reuse by the cell.
Other Names
ACOX; acyl-CoA oxidase 1, palmitoyl; acyl-CoA oxidase, straight-chain; acyl-Coenzyme A oxidase 1, palmitoyl; AOX; PALMCOX; palmitoyl-CoA oxidase; peroxisomal acyl-coenzyme A oxidase 1; peroxisomal fatty acyl-CoA oxidase; SCOX; straight-chain acyl-CoA oxidase
Related Genetics Topics
Identifiers
- NCBI Gene: 51
- OMIM: 609751
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
