Complement Factor H / CFH抗体

Complement Factor H / CFH抗体产品信息

免疫原 :
Recombinant Human Complement Factor H / CFH protein (Catalog#10714-H08H)
Antibody Type : Rabbit Monoclonal Antibody ( Rabbit mAb Service Platform )
克隆号 :
305
抗体宿主 :
Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
This antibody was obtained from a rabbit immunized with purified, recombinant Human Complement Factor H / CFH (rh Complement Factor H / CFH; Catalog#10714-H08H; NP_000177.2; Ser 860-Arg 1231).
Complement Factor H / CFH抗体 Background
Complement factor H, also known as H factor 1, and CFH, is a secreted protein which contains 20Sushi (CCP / SCR) domains. It is a large soluble glycoprotein that circulates in human plasma (at a concentration of 500-800 micrograms per milliliter). The main job of CFH is to regulate the Alternative Pathway of the complement system, ensuring that the complement system is directed towards pathogens and does not damage host tissue. CFH regulates complement activation on self cells by possessing both cofactor activity for the Factor I mediated C3b cleavage, and decay accelerating activity against the alternative pathway C3 convertase, C3bBb. CFH protects self cells from complement activation but not bacteria/viruses. Due to the central role that CFH plays in the regulation of complement, there are many clinical implications arrising from aberrant CFH activity. Defects in CFH are the cause of complement factor H deficiency (CFH deficiency) and hemolytic-uremic syndrome (HUS). Genetic variation in CFH is associated with age-related macular degeneration type 4 (ARMD4) which is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world.
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