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補(bǔ)體因子B抗體
  • 產(chǎn)品貨號(hào)：
    BN41955R
  • 中文名稱：
    補(bǔ)體因子B抗體
  • 英文名稱：
    Rabbit anti-Factor B Polyclonal antibody
  • 品牌：
    Biorigin

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  • BN41955R-50ul

    50ul

    ￥1486.00

    交叉反應(yīng)：Mouse,Rat 推薦應(yīng)用：WB,IHC-P,ELISA

  • BN41955R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Mouse,Rat 推薦應(yīng)用：WB,IHC-P,ELISA

  • BN41955R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Mouse,Rat 推薦應(yīng)用：WB,IHC-P,ELISA

產(chǎn)品描述

英文名稱Factor B
中文名稱補(bǔ)體因子B抗體
別    名AHUS4; B factor properdin; Factor B ; BF; BFD; C3 proaccelerator; C3 proactivator; C3/C5 convertase; CFAB; CFAB_HUMAN; CFB; Complement Factor B; Complement factor B Bb fragment; FB; FBI12; GBG; Glycine rich beta glycoprotein; Glycine-rich beta glycoprotein; H2 Bf; H2BF; PBF2; Properdin factor B,  
研究領(lǐng)域細(xì)胞生物  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來(lái)源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse, Rat, 
產(chǎn)品應(yīng)用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量55/81kDa
細(xì)胞定位分泌型蛋白 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Complement factor-B Bb fragment:301-400/764 
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹The complement component proteins, C3, C4 and C5, are potent anaphylatoxins that are released during complement activation. Binding of these proteins to their respective G protein-coupled receptors, C3aR, C1R and C5aR, induces proinflammatory events, such as cellular degranulation, smooth muscle contraction, arachidonic acid metabolism, cytokine release, leukocyte activation and cellular chemotaxis. Complement Factor B, also designated Properdin Factor B or PBF2, is part of the alternate pathway of the complement system and is cleaved by Factor D into two fragments: Ba and Bb. Bb combines with complement Factor 3b to produce the C3 or C5 convertase and plays a role in the differentiation and proliferation of preactivated B lymphocytes, lysis of erythrocytes, stimulation of lymphocyte blastogenesis and rapid spreading of peripheral blood monocytes. Ba is important in inhibiting the proliferation of preactivated B lymphocytes. Adipsin, also designated complement Factor D, is a serine protease that cleaves complement Factor B and may be involved in obesity. Factor H controls the function of the alternative complement pathway. FHR-1 (complement Factor H related protein 1) may play a role in lipid metabolism.

Function:
Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B-lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes.

Subunit:
Monomer.

Subcellular Location:
Secreted.

DISEASE:
Defects in CFB are a cause of susceptibility to hemolytic uremic syndrome atypical type 4 (AHUS4) [MIM:612924]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.

Similarity:
Belongs to the peptidase S1 family.
Contains 1 peptidase S1 domain.
Contains 3 Sushi (CCP/SCR) domains.
Contains 1 VWFA domain.

SWISS:
P00751

Gene ID:
629

Database links:

Entrez Gene: 629 Human

Entrez Gene: 14962 Mouse

Entrez Gene: 294257 Rat

Omim: 138470 Human

SwissProt: P00751 Human

SwissProt: P04186 Mouse

Unigene: 69771 Human

Unigene: 653 Mouse

Unigene: 109148 Rat



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