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Complement factor 8 beta Rabbit pAb (bs-13969R)  
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產品編號 bs-13969R
英文名稱 Complement factor 8 beta Rabbit pAb
中文名稱 補體C8β鏈抗體
別    名 C8b; CO8B_HUMAN; Complement component 8 beta polypeptide; Complement component 8 subunit beta; Complement component C8 beta chain.  
研究領域 細胞生物  細菌及病毒  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Mouse,Rat,Rabbit)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 61 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Complement factor 8 beta: 151-250/591 
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 This gene encodes one of the three subunits of the complement component 8 (C8) protein. C8 is composed of equimolar amounts of alpha, beta and gamma subunits, which are encoded by three separate genes. C8 is one component of the membrane attack complex, which mediates cell lysis, and it initiates membrane penetration of the complex. This protein mediates the interaction of C8 with the C5b-7 membrane attack complex precursor. In humans deficiency of this protein is associated with increased risk of meningococcal infections. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2013]

Function:
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.

Subcellular Location:
Secreted.

Post-translational modifications:
N-glycosylated; contains one or two bound glycans. Not O-glycosylated.

DISEASE:
Defects in C8B are a cause of complement component 8 deficiency type 2 (C8D2) [MIM:120960]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.

Similarity:
Belongs to the complement C6/C7/C8/C9 family.
Contains 1 EGF-like domain.
Contains 1 LDL-receptor class A domain.
Contains 1 MACPF domain.
Contains 2 TSP type-1 domains.

SWISS:
P07358

Gene ID:
732

Database links:

Entrez Gene: 732 Human

Entrez Gene: 100009385 Rabbit

Omim: 120960 Human

SwissProt: P07358 Human

SwissProt: P98137 Rabbit

Unigene: 391835 Human



產品圖片
Sample: Raji Cell (Human) Lysate at 30 ug Primary: Anti-Complement factor 8 beta (Bs-13969R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 61 kD Observed band size: 63 kD
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