Description
Product Characteristics: Monoclonal antibody 1A2 recognizes mouse complement factor H (CFH). CFH is the first regulatory protein of the alternative pathway of the complement system. There are three pathways of complement activation, classical, alternative, and lectin activation pathway. These pathways converge to form C3/C5 convertases that generate C3a, C3b,C5a, and C5b, each with substantial biologic activity. Complement regulators are necessary to prevent the injudicious production of these mediators and potential injury to self-tissue. The plasma proteins CFH, C4-binding protein and the cell membrane proteins complement receptor 1 (CR1, CD35), decay-accelerating factor (CD55), and membrane co-factor protein (CD46) all are members of the regulators of complement activation family. These proteins have natural affinity for C3b and/or C4b, which confers on them the ability to accelerate the intrinsic decay of C3/C5 convertases and/or act as co-factor for the cleavage and inactivation (i) of C3b and C4b by complement factor I (CFI). Genetic human analyses reveals a clear association of CFH with different human diseases. These include diseases of the kidney, the atypical form of Hemolytic Uremic Syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN). Furthermore, CFH is associated with age-related macular degeneration (AMD), a disease of the eye. Immunogen Mouse factor H-human IgG fusion protein
Target Information: This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus 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, Oct 2011]