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Rabbit Anti-PCB  antibody (bs-21700R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
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说明书: 50ul  100ul  
50ul/1180.00元
100ul/1980.00元
大包装/询价

产品编号 bs-21700R
英文名称 PCB
中文名称 丙酮酸羧化酶抗体
别    名 mitochondrial; OTTHUMP00000235155; OTTHUMP00000235156; PC; PCB; Pcx; PYC_HUMAN; Pyruvate carboxylase; Pyruvate carboxylase mitochondrial; Pyruvic carboxylase.  
研究领域 肿瘤  细胞生物  免疫学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,Human (predicted: Bovine,Rat)
产品应用 WB=1:500-2000, IHC-P=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 127kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCB : 21-120/1178 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 PCB (Pyruvate carboxylase) 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. PCB 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.

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.

Subunit:
Homotetramer.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Contains 1 ATP-grasp domain.
Contains 1 biotin carboxylation domain.
Contains 1 biotinyl-binding domain.
Contains 1 carboxyltransferase domain.

SWISS:
P11498

Gene ID:
5091

Database links:
UniProtKB/Swiss-Prot: P11498.2

产品图片
Sample:
Lane 1: Mouse Liver tissue lysates
Primary: Anti-PCB (bs-21700R) at 1/1000 dilution
Secondary: Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution
Predicted band size: 127 kDa
Observed band size: 127 kDa
Sample:
Lane 1: Human HepG2 cell lysates
Primary: Anti-PCB (bs-21700R) at 1/1000 dilution
Secondary: Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution
Predicted band size: 127 kDa
Observed band size: 127 kDa
Paraformaldehyde-fixed, paraffin embedded (human pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (PCB) Polyclonal Antibody, Unconjugated (bs-21700R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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