Product Description

PCB Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES6488-50, ES6488-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IHC, IF, ELISA

Alternate Names: PC, Pyruvate carboxylase, mitochondrial, Pyruvic carboxylase, PCB

Clonality: Polyclonal

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

Isotype: IgG

Target: PCB

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

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

Concentration: 1 mg/ml,

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

Expression: Epithelium, Kidney, Liver, Lung,

Observed Band (KD): 120kD

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

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

Storage and Stability: -20°C/1 year

Gene Name: PC

Protein Name: Pyruvate carboxylase mitochondrial

Mouse SwissProt Number: Q05920

Rat Gene ID: 25104

Rat SwissProt Number: P52873

Research Use Only