补体因子I轻链抗体

供应商 ：江西江蓝纯生物试剂有限公司

库存 ：159

克隆性 ：单克隆

保质期 ：1年

抗体英文名 ：Factor I light chain

抗体名 ：补体因子I轻链抗体

适应物种 ：人/动物/植物

应用范围 ：WB,ELISA等

浓度 ：1mg/ml

保存条件 ：-20 °

规格 ：100ul/200ul

产品货号 :  jlcR24314
英文名称 :  Factor I light chain
中文名称 : 补体因子I轻链抗体
别    名 :  AHUS3; ARMD13; C3b INA; C3b inactivator; C3B/C4B inactivator; C3BINA; CFAI_HUMAN; Cfi; Complement component I; Complement control protein factor I; Complement factor I; Complement factor I heavy chain; Complement factor I light chain; F1; factor I; FactorI; FI; I factor; IF; KAF; Konglutinogen activating factor; Light chain of factor I; OTTHUMP00000219728; OTTHUMP00000221928  
研究领域 :  心血管  免疫学  
抗体来源 :  Rabbit
克隆类型 :  Polyclonal
交叉反应  :  Mouse, Rat, Cow,
产品应用 :  IHC-P=1:400-800 （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 :  22kDa
细胞定位 :  细胞外基质 分泌型蛋白
性    状 :  Lyophilized or Liquid
浓    度 :  1mg/ml
免 疫 原 :  KLH conjugated synthetic peptide derived from mouse Factor I light chain :451-550/582
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.
PubMed :  PubMed
产品介绍 :   The complement pathway is an important host defense system that contributes to both innate and acquired immunity. There are three pathways of complement activation: the classical pathway, lectin pathway and alternative pathway. Complement protein Factor I is a key serine protease that modulates the complement cascade by regulating the levels of C3 convertases. It circulates in plasma as a heavily N-glycosylated heterodimer made up of two disulfide linked chains, each carrying three N-linked oligosaccharide chains that may have both structural and functional roles in the interactions with the natural substrate and the cofactor during catalysis. Factor I is a serine protease with a high degree of specificity for C3b and C4b. It requires protein cofactors for cleavage of these complement proteins; Factor H, CR1 or MCP are required for C3b cleavage, and C4bp or CR1 are required for C4b cleavage.

Function:
Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Subcellular Location : Secreted; extracellular space.

Subcellular Location:
Secreted; extracellular space.

Tissue Specificity:
Plasma.

DISEASE:
Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3) [MIM:612923]. 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. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency) [MIM:610984]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.

Similarity:
Belongs to the peptidase S1 family.
Contains 1 Kazal-like domain.
Contains 2 LDL-receptor class A domains.
Contains 1 peptidase S1 domain.
Contains 1 SRCR domain.

SWISS:
P05156

Gene ID:
3426

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

产品图片