靶标：
CFI
产品别名：
AHUS3； ARMD13； C3BINA； C3b-INA； FI； IF； KAF； CFI； complement factor I； complement factor I； complement factor I； C3B/C4B inactivator； C3b-inactivator； Konglutinogen-activating factor； complement component I； complement control protein factor I； complement factor I heavy chain； light chain of factor I； 补体因子I(CFI)； 补体因子I轻链； 补体因子I重链；
背景信息：
This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by Ref
宿主：Rbt
类型：Pab
同种型:IgG
应用：WB
纯化方式：亲和纯化
偶联物：Unconjugated
性状：液体
存储溶液：参阅说明书
浓度：Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user
储存：经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项：仅供实验室使用。不适用于人类或动物的任何临床，治疗或诊断用途。不适合动物或人类食用。
