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. |