Alpha-methylacyl-CoA racemase

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Ideogram human chromosome 5

Alpha-methylacyl-CoA racemase

Alpha-methylacyl-CoA racemase (AMACR) is an enzyme that plays a crucial role in fatty acid metabolism. It is encoded by the AMACR gene located on chromosome 5. AMACR is involved in the breakdown of branched-chain fatty acids and bile acid intermediates. This enzyme is primarily found in the liver, kidney, and prostate tissues.

Function[edit | edit source]

AMACR catalyzes the racemization of alpha-methyl-branched-chain fatty acids, converting the D-isomer to the L-isomer. This process is essential for the degradation of these fatty acids and subsequent energy production. Additionally, AMACR is involved in bile acid synthesis by converting intermediates into the final bile acids.

Clinical Significance[edit | edit source]

Mutations in the AMACR gene have been associated with certain metabolic disorders, including alpha-methylacyl-CoA racemase deficiency. This condition can lead to the accumulation of toxic metabolites and impaired fatty acid metabolism. Furthermore, AMACR expression has been linked to prostate cancer, where increased levels of this enzyme are observed in malignant prostate tissues.

Diagnostic and Therapeutic Implications[edit | edit source]

AMACR expression is used as a diagnostic marker for prostate cancer, as its overexpression is a common feature of prostate tumors. In addition, inhibitors targeting AMACR activity are being explored as potential therapeutic agents for prostate cancer treatment. Understanding the role of AMACR in fatty acid metabolism may also provide insights into metabolic disorders and potential therapeutic interventions.

See also[edit | edit source]


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Contributors: Prab R. Tumpati, MD