alpha-methylacyl-CoA racemase (2-methylacyl-CoA racemase, AMACR)

Function

• carbohydrate metabolism
• bile acid biosynthesis
• lipid metabolism, fatty acid metabolism
• racemization of 2-methyl-branched fatty acid CoA esters
• responsible for the conversion of pristanoyl-CoA & C27-bile acyl-CoAs to their (S)-stereoisomers

(2S)-2-methylacyl-CoA <--> (2R)-2-methylacyl-CoA

Structure

• belongs to the caiB/baiF CoA-transferase family

Compartment

peroxisome, mitochondrion

Pathology

• defects in AMACR are the cause of AMACR deficiency

Laboratory

• alpha-methylacyl CoA racemase in fibroblasts

More general terms

• isomerase
• mitochondrial protein

Database

• OMIM: https://mirror.omim.org/entry/604489
• UniProt: http://www.uniprot.org/uniprot/Q9UHK6.html