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折扣/货期| 货号：GTX41627 | 规格：100μg | 目录价：￥10700 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
Factor I antibody [3R/8]
别名:
complement factor I , AHUS3 , ARMD13 , C3BINA , C3b-INA , FI , IF , KAF
反应种属:
Human
宿主来源:
Mouse
实验应用:
ELISA, IHC-Fr, Neutralizing /Inhibition, WB
靶标/特异性:
This antibody recognizes complement factor I. Clone 3R/8 blocks function of complement factor I.
同种型:
IgG1
免疫原:
Native human factor I from serum.
克隆性:
Monoclonal
克隆号:
3R/8
纯化方式:
Protein A purified
偶联:
Unconjugated
产品浓度:
1.0 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
Borate buffered saline, 0.1% Sodium azide.
生产商:
GeneTex
功能与背景:
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. [provided by RefSeq, Dec 2015]
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