Complement Factor I 抗体
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- 抗原 See all Complement Factor I (CFI) 抗体
- Complement Factor I (CFI)
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适用
- 人, 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This Complement Factor I antibody is un-conjugated
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应用范围
- Immunofluorescence (IF)
- 产品特性
- Polyclonal Antibody
- 纯化方法
- Affinity purification
- 免疫原
- Recombinant fusion protein of human CFI (NP_000195.2).
- 亚型
- IgG
- Top Product
- Discover our top product CFI Primary Antibody
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- 应用备注
- IF 1:50-1:100
- 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 mg/mL
- 缓冲液
- PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3
- 储存液
- Sodium azide
- 注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Avoid freeze / thaw cycles.
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- 抗原
- Complement Factor I (CFI)
- 别名
- CFI (CFI 产品)
- 别名
- cfi antibody, MGC53615 antibody, Cfi antibody, factor I antibody, IF antibody, gb:ai721528 antibody, ahus3 antibody, c3b-ina antibody, c3bc4bi antibody, c3bina antibody, kaf antibody, CFI antibody, AHUS3 antibody, C3BINA antibody, C3b-INA antibody, FI antibody, KAF antibody, complement factor I S homeolog antibody, complement factor I L homeolog antibody, complement factor I antibody, complement component factor i antibody, cfi.S antibody, cfi.L antibody, CFI antibody, cfi antibody, Cfi antibody
- 背景
- 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.
- 基因ID
- 3426
- UniProt
- P05156
- 途径
- Complement System
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