FITC标记的补体因子I轻链抗体上海沪震实业有限公司

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产品名称: FITC标记的补体因子I轻链抗体
英文名称: Anti-Factor I light chain/FITC
抗体货号: HZ-13131R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/9/14 10:53:00
详细资料:  实验方法技术资料
浏览人数:7
诚信指数:1084点
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
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上海·中国-上海
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产品详细描述

 Rabbit Anti-Factor I light chain/FITC Conjugated antibody 

FITC标记的补体因子I轻链抗体

 

英文名称 Anti-Factor I light chain/FITC
中文名称 FITC标记的补体因子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  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Cow, Horse, 
产品应用 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 22kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Factor I Complement factor I light chain
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 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.

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.

Database links:

 

Entrez Gene: 3426 Human

Omim: 217030 Human

SwissProt: P05156 Human

Unigene: 312485 Human

 



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

补体途径是一种重要的宿主防御系统,有助于先天免疫和后天免疫。补体激活有三条途径:经典途径、凝集素途径和替代途径。补体蛋白因子I是一种重要的丝氨酸蛋白酶,通过调节C3转化酶的水平来调节补体级联。它在血浆中循环为由两个二硫键连接的链组成的重N-糖基化异二聚体,每个链带有三个N-键连接的低聚糖链,它们在催化过程中可能具有与天然底物和辅因子相互作用的结构和功能作用。因子I是丝氨酸蛋白酶,对C3b和C4b具有高度特异性,需要蛋白辅因子来切割这些互补蛋白,C3b切割需要因子H、CR1或MCP,C4b切割需要C4bp或CR1。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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