Alpha-methylacyl-CoA racemase

AMACR
Identifiers
Aliases	AMACR, AMACRD, CBAS4, RACE, RM, alpha-methylacyl-CoA racemase, P504S
External IDs	OMIM: 604489; MGI: 1098273; HomoloGene: 7410; GeneCards: AMACR; OMA:AMACR - orthologs
EC number	5.1.99.4
Gene location (Human)
Chr.	Chromosome 5 (human)
End	34,008,104 bp
Gene location (Mouse)
Chr.	Chromosome 15 (mouse)
End	10,996,712 bp
RNA expression pattern
	Top expressed in
	rectum; ; liver; ; human kidney; ; mucosa of transverse colon; ; right lobe of liver; ; islet of Langerhans; ; Achilles tendon; ; fundus; ; renal cortex; ; gallbladder;
	Top expressed in
	right kidney; ; left lobe of liver; ; proximal tubule; ; human kidney; ; myocardium of ventricle; ; intercostal muscle; ; digastric muscle; ; sternocleidomastoid muscle; ; cardiac muscles; ; triceps brachii muscle;
	More reference expression data
	n/a
Gene ontology
Molecular function	signaling receptor binding; catalytic activity; isomerase activity; alpha-methylacyl-CoA racemase activity;
Cellular component	cytoplasm; peroxisome; peroxisomal matrix; mitochondrion; plasma membrane; intracellular membrane-bounded organelle; cytosol;
Biological process	bile acid biosynthetic process; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid metabolic process; bile acid metabolic process; protein targeting to peroxisome;
	Sources:Amigo / QuickGO
Orthologs
	23600
	17117
	ENSG00000242110
	ENSMUSG00000022244
	Q9UHK6
	O09174
NM_203382; NM_001167595; NM_001167596; NM_001167597; NM_001167598;
	NM_014324
	NM_008537
	NP_001161067; NP_055139; NP_976316
	NP_032563
	Wikidata
View/Edit Human	View/Edit Mouse

α-Methylacyl-CoA racemase (AMACR, EC 5.1.99.4) is an enzyme that in humans is encoded by the AMACR gene. AMACR catalyzes the following chemical reaction:

(2R)-2-methylacyl-CoA

\rightleftharpoons

(2S)-2-methylacyl-CoA

In mammalian cells, the enzyme is responsible for converting (2R)-methylacyl-CoA esters to their (2S)-methylacyl-CoA epimers and known substrates, including coenzyme A esters of pristanic acid (mostly derived from phytanic acid, a 3-methyl branched-chain fatty acid that is abundant in the diet) and bile acids derived from cholesterol. This transformation is required in order to degrade (2R)-methylacyl-CoA esters by β-oxidation, which process requires the (2S)-epimer. The enzyme is known to be localised in peroxisomes and mitochondria, both of which are known to β-oxidize 2-methylacyl-CoA esters.