α-methylacyl-CoA Racemase (AMACR) catalyzes the racemization of α-methyl-branched carboxylic acid coenzyme A thioesters. This enzymatic function is involved with biosynthesis of bile acids in the mitochondria and peroxisomes. AMACR converts pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers, which are the only stereoisomers degraded by peroxisomal α-oxidation. Interestingly, AMACR mutations have been linked to some sensory motor neuropathies where accumulation of fatty acids and AMACR deficiencies correlate with pathogenesis. AMACR contains an N-terminal region required for mitochondrial localization, and a C-terminal peroxisomal targeting signal type 1 (PTS). AMACR mRNA is expressed preferentially in human, rat, and mouse liver and kidney, but the percentage of AMACR enzymatic activity found in the mitochondria relative to the peroxisomes differs depending on the species. Thus, AMACR is an enzyme critical for fatty acid degradation, and bile formation.
原厂资料：
注意事项：
1.Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2.Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
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