Citramalyl-CoA lyase, mitochondrial

Definition

Mitochondrial citramalyl-CoA lyase indirectly involved in the vitamin B12 metabolism. Converts citramalyl-CoA into acetyl-CoA and pyruvate in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite. Also acts as a malate synthase in vitro, converting glyoxylate and acetyl-CoA to malate. Also displays malyl-CoA thioesterase activity. Also acts as a beta-methylmalate synthase in vitro, by mediating conversion of glyoxylate and propionyl-CoA to beta-methylmalate. Also has very weak citramalate synthase activity in vitro.

References:

[1]. Hongying Shen, et al. The Human Knockout Gene CLYBL Connects Itaconate to Vitamin B12. Cell. 2017 Nov 2;171(4):771-782.e11. [Content Brief]

[2]. Laura Strittmatter, et al. CLYBL is a polymorphic human enzyme with malate synthase and β-methylmalate synthase activity. Hum Mol Genet. 2014 May 1;23(9):2313-23. [Content Brief]

生物医学辞典

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