Molecular weight (DA)
120kD
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.
Dilution rate
Western Blot: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000. IF 1:100-300 Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Pyruvate carboxylase mitochondrial
Other name
PC; Pyruvate carboxylase; mitochondrial; Pyruvic carboxylase; PCB
Fields
>>Citrate cycle (TCA cycle);>>Pyruvate metabolism;>>Metabolic pathways;>>Carbon metabolism;>>Biosynthesis of amino acids
Human protein sequence Database
P11498
Mouse protein sequence database
Q05920
Rat protein sequence database
P52873
Cellular localization
Mitochondrion matrix .
Tissue expression
Epithelium,Kidney,Liver,Lung,
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