Kids Kidney Research

The pivotal role of the complement system in atypical HUS (aHUS) has recently been recognised.  Complement is an ancient defence system which coats both foreign cells (such as bacteria and viruses) and our own cells (including those that line the blood vessels in the kidney) with a substance called C3b.  This substance will eventually lead to the destruction of bacteria and viruses by damaging the cell wall.  If our own cells are not protected against C3b they can suffer a similar fate.  This is why we have a series of regulators which inactivate C3b.  We have found that 10% of patients with aHUS have antibodies against one of these regulators called factor H which impair its function.  In this study we wish to examine whether aHUS patients have antibodies against other regulators and if so what they do to the function of the regulators.  There are now specific treatments for antibody associated diseases and this opens up another possible avenue of treatment for aHUS patients.