CLYBL


Description

The CLYBL (citramalyl-CoA lyase) is a protein-coding gene located on chromosome 13.

CLYBL, also known as citramalyl-CoA lyase, is a mitochondrial enzyme involved in the detoxification of itaconate, a vitamin B12-poisoning metabolite. It catalyzes the conversion of citramalyl-CoA into acetyl-CoA and pyruvate in the C5-dicarboxylate catabolism pathway. Additionally, CLYBL acts as a malate synthase, converting glyoxylate and acetyl-CoA to malate, and displays malyl-CoA thioesterase activity. In vitro, CLYBL can also function as a beta-methylmalate synthase, converting glyoxylate and propionyl-CoA to beta-methylmalate, and exhibits very weak citramalate synthase activity.

CLYBL is also known as CLB.

Associated Diseases


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