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Rabbit Anti-Complement factor 8 beta/BF594 Conjugated antibody (bs-13969R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13969R-BF594
英文名稱 Rabbit Anti-Complement factor 8 beta/BF594 Conjugated antibody
中文名稱 BF594標記的補體C8β鏈抗體
別    名 C8b; CO8B_HUMAN; Complement component 8 beta polypeptide; Complement component 8 subunit beta; Complement component C8 beta chain.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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
交叉反應 (predicted: Human, Mouse, Rat, Rabbit, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 61kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Complement factor 8 beta
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
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.

Database links:

Entrez Gene: 732 Human

Entrez Gene: 100009385 Rabbit

Omim: 120960 Human

SwissProt: P07358 Human

SwissProt: P98137 Rabbit

Unigene: 391835 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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