1. Molecular defects in C regulation associated with C3 glomerulopathy and atypical hemolytic uremic syndrome.
Molecular defects in C regulation associated with C3 glomerulopathy and atypical hemolytic uremic syndrome. In health, factor H controls C activation in the fluid phase and on the glomerular basement membrane. Factor H and cell surface C regulatory proteins (MCP, membrane cofactor protein; DAF, decay accelerating factor; THBD, thrombomodulin) control C activation on glomerular endothelial cells. In patients with C3 glomerulopathy, congenital or acquired defects probably cause dysregulated C activation in the fluid phase and/or on the glomerular basement membrane. In atypical hemolytic uremic syndrome, congenital or acquired defects probably cause dysregulated C activation on glomerular endothelial cells. Defects in C regulation in the different conditions are indicated with red boxes or red font.
Joshua M. Thurman, and Carla M. Nester CJASN 2016;11:
©2016 by American Society of Nephrology