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

General

isomerase mitochondrial protein

Properties

SIZE: MW = 42 kD entity length = 382 aa COMPARTMENT: mitochondria MOTIF: microbody-targeting signal {380-382}

Database Correlations

OMIM 604489 UniProt Q9UHK6 Pfam PF02515 ENZYME 5.1.99.4

References

UniProt :accession Q9UHK6