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Description_x000D_
General description_x000D_
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)_x000D_
Immunogen_x000D_
CFH (NP_001014975.1, 1 a.a. ~ 449 a.a) full-length human protein._x000D_
Sequence_x000D_
MRLLAKIICLMLWAICVAEDCNELPPRRNTEILTGSWSDQTYPEGTQAIYKCRPGYRSLGNVIMVCRKGEWVALNPLRKCQKRPCGHPGDTPFGTFTLTGGNVFEYGVKAVYTCNEGYQLLGEINYRECDTDGWTNDIPICEVVKCLPVTAPENGKIVSSAMEPDREYHFGQAVRFVCNSGYKIEGDEEMHCSDDGFWSKEKPKCVEISCKSPDVINGSPISQKIIYKENERFQYKCNMGYEYSERGDAVCTESGWRPLPSCEEKSCDNPYIPNGDYSPLRIKHRTGDEITYQCRNGFYPATRGNTAKCTSTGWIPAPRCTLKPCDYPDIKHGGLYHENMRRPYFPVAVGKYYSYYCDEHFETPSGSYWDHIHCTQDGWSPAVPCLRKCYFPYLENGYNQNHGRKFVQGKSIDVACHPGYALPKAQTTVTCMENGWSPTPRCIRVSFTL_x000D_
Physical form_x000D_
Solution in phosphate buffered saline, pH 7.4_x000D_
Disclaimer_x000D_
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._x000D_
Biochem/physiol Actions_x000D_
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.
- Related Categories Alphabetical Index, Antibodies, CE-CH, Primary Antibodies conjugate