AMACR : alpha-methylacyl-CoA racemase
Description
The AMACR (alpha-methylacyl-CoA racemase) is a protein-coding gene located on chromosome 5.
The AMACR gene provides instructions for making the enzyme alpha-methylacyl-CoA racemase (AMACR). This enzyme is found in mitochondria and peroxisomes. Peroxisomes break down various substances, including fatty acids and toxic compounds, and are important for lipid synthesis. In peroxisomes, AMACR helps break down pristanic acid, a fatty acid found in meat and dairy products. In mitochondria, AMACR further breaks down molecules derived from pristanic acid.
AMACR catalyzes the conversion of (R)- and (S)-stereoisomers of alpha-methyl-branched-chain fatty acyl-CoA esters. It only acts on coenzyme A thioesters, not on free fatty acids. AMACR can process a variety of alpha-methylacyl-CoAs, including pristanoyl-CoA, trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), and arylpropionic acids like ibuprofen. However, it does not process 3-methyl-branched or linear-chain acyl-CoAs.
AMACR is also known as AMACRD, CBAS4, P504S, RACE, RM.
Associated Diseases
- Congenital bile acid synthesis defect type 4
- Bile acid synthesis defect, congenital, 4
- Alpha-methylacyl-CoA racemase deficiency
