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Complement factor H, also known as Factor H 1 and CFH, is a glycoprotein containing sialic acid that plays an integral role in the regulation of the complement mediated immune system, which is involved in microbial defense, immune complex processing, and programmed cell death. CFH acts as a regulator of alternative pathways in the complement system by ensuring that the associated immune response acts on foreign pathogens that are not host cells. CFH protects its own cells from complement activation, but not bacteria and viruses. Misregulation of CFH may adversely affect the ability to deal with foreign infections or reduce the complement activity of host cells. Mutations in the Factor H gene have been linked to a variety of serious diseases, including the rare kidney disease hemolytic uremic syndrome (HUS) and membranous proliferative glomerulonephritis (MPGN), also known as dense matter deposition disease (DDD), membranous proliferative glomerulonephritis type II or dense matter deposition disease, and the more common age-related macular degeneration (AMD).
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