How is pathogenesis of C3 glomerulopathy?

➢Complement dysregulation due to acquired or hereditary abnormalities of the alternative pathway of complement activation is the underlying cause of dense deposit disease and C3 GN. ➢Some patients have an autoantibody against C3 convertase, called C3 nephritic factor (C3NeF), that causes uncontrolled cleavage of C3 by the alternative complement pathway. ➢ In other patients, mutations in various complement regulatory proteins, such as Factor H, Factor I, and membrane cofactor protein (MCP), or autoantibodies to Factor H, are the cause of unregulated activation of the alternative pathway of complement