CPT1 converts palmitoyl-CoA to palmitoyl carnitine Authored: Gopinathrao, G, 2007-07-29 21:03:03 Carnitine palmitoyl transferase 1 (CPT1) associated with the inner mitochondrial membrane, catalyzes the reaction of palmitoyl-CoA from the cytosol with carnitine in the mitochondrial intermembrane space to form palmitoylcarnitine and CoASH. Two CPT1 isoforms exist, encoded by two different genes. In the body, CPT1A is most abundant in liver while CPT1B is abundant in muscle. Both CPT1A and CPT1B are inhibited by malonyl-CoA (Morillas et al. 2002, 2004; Zammit et al. 2001; Zhu et al. 1997). Mutations in CPT1A are associated with defects in fatty acid metabolism and fasting intoilerance, consistent with the role assigned to CPT1 from studies in vitro and in animal models (IJlst et al. 1998; Gobin et al. 2003). EC Number: 2.3.1.21 Pubmed11356169 Pubmed11790793 Pubmed14517221 Pubmed14711372 Pubmed9344464 Pubmed9691089 Reactome Database ID Release 43200406 Reactome, http://www.reactome.org ReactomeREACT_11185 Reviewed: D'Eustachio, P, 2007-07-31 18:50:15 palmitoyl-CoA + carnitine => palmitoylcarnitine + CoASH