Pyruvate carboxylase: Difference between revisions
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==Function== | ==Function== | ||
Pyruvate carboxylase (PC) encoded by the gene PC is an enzyme (EC 6.4.1.1) of the ligase class that catalyzes (depending on the species) the physiologically irreversible carboxylation of pyruvate to form oxaloacetate (OAA). PC is a biotin-containing enzyme. PC is a tetrameric protein containing biotin carboxylase, carboxyltransferase, allosteric effector (acetyl-CoA) and biotin carboxyl carrier domains. PC is regulated by acetyl-CoA and Asp. The biotin moiety transfers the carboxyl group from the biotin carboxylase active site to the carboxyltransferase active site<ref>PMID:18613815</ref> . | Pyruvate carboxylase (PC) encoded by the gene PC is an enzyme (EC 6.4.1.1) of the ligase class that catalyzes (depending on the species) the physiologically irreversible carboxylation of pyruvate to form oxaloacetate (OAA). PC is a biotin-containing enzyme. PC is a tetrameric protein containing biotin carboxylase (BC), carboxyltransferase (CT), allosteric effector (acetyl-CoA) and biotin carboxyl carrier protein (BCCP) domains. PC is regulated by acetyl-CoA and Asp. The biotin moiety transfers the carboxyl group from the biotin carboxylase active site to the carboxyltransferase active site<ref>PMID:18613815</ref> . | ||
==Disease== | ==Disease== | ||
Mutations in the PC gene cause 3 types of clinical spectra. Type A and B are neonatal forms causing early death; type C causes mild intellectual delay<ref>PMID:30870574</ref> . | Mutations in the PC gene cause 3 types of clinical spectra. Type A and B are neonatal forms causing early death; type C causes mild intellectual delay<ref>PMID:30870574</ref> . Deregulation of PC expression is associated with type 2 diabetics and tumorgenesis in several cancers<ref>PMID:29362846</ref> . | ||
==Structural highlights== | ==Structural highlights== | ||
The 3D structure of PC shows its 4 domains: BC, CT, allosteric effector and BCCP. The ATP moiety active site is in the BC domain and contains 2 Mg++ ions. The effector CoA is bound to the BC domain and the allosteric effector domain<ref>PMID:17717183</ref> . | |||
==3D structures of pyruvate carboxylase== | ==3D structures of pyruvate carboxylase== | ||
Revision as of 08:41, 11 January 2023
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