[HTML][HTML] Disease-associated N-terminal complement factor H mutations perturb cofactor and decay-accelerating activities
IC Pechtl, D Kavanagh, N Mcintosh, CL Harris… - Journal of Biological …, 2011 - ASBMB
Many mutations associated with atypical hemolytic uremic syndrome (aHUS) lie within
complement control protein modules 19–20 at the C terminus of the complement regulator
factor H (FH). This region mediates preferential action of FH on self, as opposed to foreign,
membranes and surfaces. Hence, speculation on disease mechanisms has focused on
deficiencies in regulation of complement activation on glomerular capillary beds. Here, we
investigate the consequences of aHUS-linked mutations (R53H and R78G) within the FH N …
complement control protein modules 19–20 at the C terminus of the complement regulator
factor H (FH). This region mediates preferential action of FH on self, as opposed to foreign,
membranes and surfaces. Hence, speculation on disease mechanisms has focused on
deficiencies in regulation of complement activation on glomerular capillary beds. Here, we
investigate the consequences of aHUS-linked mutations (R53H and R78G) within the FH N …