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货号： bs-6949R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-6949R-Bio
- 英文名称
- Anti-Factor B/Biotin
- 中文名称
- 生物素标记的补体因子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,
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- Human, Mouse, Rat,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=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
- 55/81kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Complement factor-B Bb fragment
- 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
- 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.
- 产品介绍
- background:
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.
Database links:
UniProtKB/Swiss-Prot: P00751.2
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.