|Other Names||Complement factor H-related protein 5, FHR-5, CFHR5, CFHL5, FHR5|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH.|
|Tissue Location||Expressed by the liver and secreted in plasma.|
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CFHR5 is a member of a small complement factor H (CFH)gene cluster on chromosome 1. Each member of this gene familycontains multiple short consensus repeats (SCRs) typical ofregulators of complement activation. The protein encoded by thisgene has nine SCRs with the first two repeats having heparinbinding properties, a region within repeats 5-7 having heparinbinding and C reactive protein binding properties, and theC-terminal repeats being similar to a complement component 3 b(C3b) binding domain. This protein co-localizes with C3, binds C3bin a dose-dependent manner, and is recruited to tissues damaged byC-reactive protein. Allelic variations in this gene have beenassociated, but not causally linked, with two different forms ofkidney disease: membranoproliferative glomerulonephritis type II(MPGNII) and hemolytic uraemic syndrome (HUS). [provided byRefSeq].
McRae, J.L., et al. Genetica 114(2):157-161(2002)Murphy, B., et al. Am. J. Kidney Dis. 39(1):24-27(2002)Narkio-Makela, M., et al. Clin. Immunol. 100(1):118-126(2001)McRae, J.L., et al. J. Biol. Chem. 276(9):6747-6754(2001)
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