Target：PCB

Fields：Citrate cycle (TCA cycle);Pyruvate metabolism;Metabolic pathways;Carbon metabolism;Biosynthesis of amino acids

Gene Name：PC

Protein Name：Pyruvate carboxylase mitochondrial

Human Gene Id：5091

Human Swiss Prot No：P11498

Mouse Swiss Prot No：Q05920

Rat Gene Id：25104

Rat Swiss Prot No：P52873

Immunogen：The antiserum was produced against synthesized peptide derived from human PC. AA range:357-406

Specificity：PCB Polyclonal Antibody detects endogenous levels of PCB protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC: 1:100-300 ELISA: 1:20000. IF 1:100-300 Not yet tested in other applications.

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：PC;Pyruvate carboxylase; mitochondrial;Pyruvic carboxylase;PCB

Observed Band(KD)：120kD

Background： This gene encodes pyruvate carboxylase, which requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. Pyruvate carboxylase is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency. Alternatively spliced transcript variants with different 5' UTRs, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008],

Function：catalytic activity:ATP + pyruvate + HCO(3)(-) = ADP + phosphate + oxaloacetate.,cofactor:Binds 1 manganese ion per subunit.,cofactor:Biotin.,disease:Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia.,function:Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.,online information:Pyruvate carboxylase entry,pathway:Carbohydrate biosynthesis; gluconeogenesis.,similarity:Conta

Subcellular Location：Mitochondrion matrix .

Expression： Epithelium,Kidney,Liver,Lung,