Search
pyruvate carboxylase, mitochondrial (pyruvic carboxylase, PCB, PC)
Function:
- gluconeogenesis & lipogenesis
- catalyzes the ATP-dependent reaction of pyruvate with CO2 to form oxaloacetate
- pyruvate carboxylase catalyzes a 2-step reaction, involving
a) ATP-dependent carboxylation of covalently attached biotin
b) transfer of the carboxyl group to pyruvate
- catalyzes (tissue specific) initial reactions of glucose (liver, kidney) & lipid (adipose tissue, liver, brain) synthesis from pyruvate
ATP + pyruvate + HCO3- ADP + phosphate + oxaloacetate
Cofactors: biotin, Mn+2
Structure:
- homotetramer
- contains 1 ATP-grasp domain
- contains 1 biotin carboxylation domain
- contains 1 biotinyl-binding domain
- contains 1 carboxyltransferase domain
Compartment: mitochondrial matrix
Pathology:
- defects in PC result in pyruvate carboxylase deficiency
Interactions
molecular events
General
carboxy-lyase or decarboxylase
gluconeogenic enzyme
mitochondrial protein
phosphoprotein
Properties
SIZE: entity length = 1178 aa
MW = 130 kD
COMPARTMENT: mitochondria
MOTIF: Biotin carboxylation {36-486}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 152-152
ATP-grasp domain
SITE: 156-353
ATP-binding site
NAME: ATP-binding site
SITE: 236-236
ATP-binding site
NAME: ATP-binding site
SITE: 271-271
arginine residue {R328}
Carboxyltransferase {563-832}
Tyr phosphorylation site {Y894}
binding site
SITE: 1110-1177
FOR-BINDING-OF: BIOTINYL
MOTIF: binding site
SITE: 1144-1144
FOR-BINDING-OF: Biotin (covalent)
Database Correlations
OMIM correlations
MORBIDMAP 608786
UniProt P11498
PFAM correlations
ENZYME 6.4.1.1
References
- UniProt :accession P11498
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PC
- Wikipedia; pyruvate carboxylase entry
http://en.wikipedia.org/wiki/pyruvate_carboxylase