Anti-Factor I antibody
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概述
- 产品描述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
- 产品名称Anti-Factor I antibody
- 分子量66 kDa
- 种属反应性Human
- 验证应用WB
- 抗体类型兔多抗
- 免疫原The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490
- 偶联Non-conjugated
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性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
- 存储缓冲液Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 亚型IgG
- 纯化方式Immunogen affinity purified.
- 其它名称CFI antibody; IF antibody; Complement factor I antibody; C3B/C4B inactivator antibody;
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