产品名称
PCB Rabbit Polyclonal Antibody
别名
PC; Pyruvate carboxylase; mitochondrial; Pyruvic carboxylase; PCB
蛋白名称
Pyruvate carboxylase mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5091
Human Swissprot No.
P11498
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P11498/entry
Mouse Swissprot No.
Q05920
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q05920
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=25104
Rat Swissprot Link
http://www.uniprot.org/uniprot/P52873
免疫原
The antiserum was produced against synthesized peptide derived from human PC. AA range:357-406
特异性
PCB Polyclonal Antibody detects endogenous levels of PCB protein.
稀释度
WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:20000. IF 1:100-300 Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Epithelium,Kidney,Liver,Lung,
细胞定位
Mitochondrion matrix .
信号通路
Citrate cycle (TCA cycle);Pyruvate metabolism;
功能
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:Contains 1 ATP-grasp domain.,similarity:Contains 1 biotin carboxylation domain.,similarity:Contains 1 biotinyl-binding domain.,similarity:Contains 1 carboxyltransferase domain.,subunit:Homotetramer.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
