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Rabbit Anti-PCB  antibody (bs-21700R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-21700R
英文名稱 Rabbit Anti-PCB  antibody
中文名稱 丙酮酸羧化酶抗體
別    名 PYC_HUMAN; Pyruvate carboxylase, mitochondrial; EC:6.4.1.1; Pyruvic carboxylase (PCB); PCB; Pcx;  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse (predicted: Rat,Bovine)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 127kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCB: 21-120/1178 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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

產品圖片
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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