Anti-CFH antibody produced in rabbit

产品说明

一般描述

This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short concensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. (provided by RefSeq)

免疫原

CFH (NP_001014975.1, 1 a.a. ~ 449 a.a) full-length human protein.

Sequence
MRLLAKIICLMLWAICVAEDCNELPPRRNTEILTGSWSDQTYPEGTQAIYKCRPGYRSLGNVIMVCRKGEWVALNPLRKCQKRPCGHPGDTPFGTFTLTGGNVFEYGVKAVYTCNEGYQLLGEINYRECDTDGWTNDIPICEVVKCLPVTAPENGKIVSSAMEPDREYHFGQAVRFVCNSGYKIEGDEEMHCSDDGFWSKEKPKCVEISCKSPDVINGSPISQKIIYKENERFQYKCNMGYEYSERGDAVCTESGWRPLPSCEEKSCDNPYIPNGDYSPLRIKHRTGDEITYQCRNGFYPATRGNTAKCTSTGWIPAPRCTLKPCDYPDIKHGGLYHENMRRPYFPVAVGKYYSYYCDEHFETPSGSYWDHIHCTQDGWSPAVPCLRKCYFPYLENGYNQNHGRKFVQGKSIDVACHPGYALPKAQTTVTCMENGWSPTPRCIRVSFTL

生化/生理作用

CFH (complement factor H) is a negative regulator of alternate pathway. It is known to suppress spontaneous complement activation on self surfaces alone, and is therefore surface selective. CFH possesses decay-accelerating activity and destabilizes C3b amplification in the alternate complement pathway. CFH is responsible for C3b cleavage by its cofactor activity. CFH is a membrane cofactor protein, variation in which is associated with a number of diseases such as age-related macular degeneration, kidney disorder and C3 glomerulopathy. The penetrance is found to be low in many diseases. CFH might prevent complement activation in ischaemic renal injury by binding to tubular epithelial cells.

外形

Solution in phosphate buffered saline, pH 7.4

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

产品性质

安全信息