Anti-CFI Polyclonal Antibody-一抗-抗体-蛋白与免疫

Anti-CFI Polyclonal Antibody

品牌:solarbio | 货号:K004089P

英文名称 Anti-CFI Polyclonal Antibody
宿主 Rabbit
别名 AHUS3;ARMD13;C3BINA;C3b-INA;FI;IF;KAF
应用 WB IF
稀释比例 WB 1:500-2000. IF 1:50-100.
交叉反应 Human Mouse
蛋白分子量 66kDa
Gene ID 3426
保存 Store at -20°C. Avoid freeze / thaw cycles.
储存液 Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
纯化方法 Affinity purification
亚型 IgG
免疫原 Recombinant protein of human CFI
性状 液体
Public Immunogen Range Recombinant protein of human CFI
Subcellular Locations Secreted extracellular space
Swiss Prot P05156
克隆类型 Polyclonal Antibody
背景资料 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.