补体因子B抗体

补体因子B抗体

价格: ¥1200

品牌:Biorigin

货号:BN41955R

收藏

产品详情

相关推荐

适应物种 :Human,Mouse,Rat,

应用范围 :WB,IHC-P,

抗体英文名 :Factor B

规格 :50ul

英文名称 Factor B
中文名称 补体因子B抗体
别    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,  

 

 

研究领域 细胞生物  免疫学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse, Rat, 
产品应用 WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55/81kDa
细胞定位 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Complement factor-B Bb fragment:301-400/764 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 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.
PubMed PubMed
产品介绍 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




 

北京百瑞极生物科技有限公司

实名认证

金牌会员

入驻年限:5年

联系人:Biorigin

地址:北京

电话联系

购买咨询

换一个