品质保障》

产品介绍background:
alpha-methylacyl-CoA racemase(AMACR/P504S) is Prostate-specific antigen (PSA) screening for prostate cancer is now widespread in the United States among men of all ages. However PSA has limited specificity because benign disease, including prostatic enlargement and inflammation, can increase PSA levels. Thus, a more specific prostate cancer markers is needed. One such potential marker is AMACR, an enzyme that is involved in peroxisomal beta-oxidation of dietary branched-chain fatty acids. Recent studies have shown that, compared with expression in normal or benign prostate epithelium, AMACR is consistently overexpressed in prostate cancer epithelium, making it a specific marker for cancer cells within the prostate gland. Furthermore, overexpression of AMACR may increase the risk of prostate cancer because its expression is increased in premalignant lesions (prostatic intraepithelial neoplasia).

Function:
Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers.

Subcellular Location:
Peroxisome. Mitochondrion.

DISEASE:
Alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:614307]: A rare autosomal recessive peroxisomal disorder characterized by elevated plasma concentrations of pristanic acid C27-bile-acid intermediates, and adult onset of variable neurodegenerative symptoms affecting the central and peripheral nervous systems. Features may include seizures, visual failure, sensorimotor neuropathy, spasticity, migraine, and white matter hyperintensities on brain imaging. Note=The disease is caused by mutations affecting the gene represented in this entry.
Congenital bile acid synthesis defect 4 (CBAS4) [MIM:214950]: A disorder characterized by the presence of trihydroxycoprostanic acid in the bile and absence of cholic acid. Patients manifest neonatal jaundice, intrahepatic cholestasis and bile duct deficiency. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the CaiB/BaiF CoA-transferase family.