The understanding how complement relates to glomerular diseases has evolved considerably during the last years. Substantial evidence has accumulated that explain how a defective or deregulated complement system results in kidney diseases. The combination and close interaction of basic research with clinical medicine has demonstrated an important role of complement effector and regulatory proteins in pathological settings of the kidney. A large panel of distinct human kidney diseases such as hemolytic uremic syndrome (HUS), membrano proliferative glomerulonephritis (MPGN), systemic lupus erythematosus (SLE) and in ischemic reperfusions injury and transplantation are caused by defective complement control. Genetic analyses have identified mutations in complement regulators that are associated with these diseases. Mutations have been identified in the fluid phase alternative pathway regulator Factor H and the membrane regulator Membrane Cofactor Protein MCP (CD46). The functional characterization of the mutant proteins allows to define the pathophysiological events on a molecular level. These new concepts and data on disease mechanisms already allowed to establish new diagnostic and novel promising therapeutic approaches for several human kidney diseases.

Zusammenfassung

It is evident that a defective or deregulated complement system results in kidney diseases. An important role of complement effector and regulatory proteins in pathological settings of the kidney has been demonstrated. A large panel of distinct human kidney diseases is caused by defective complement control. Genetic analyses have identified mutations in complement regulators that are associated with these diseases. Mutations have been identified in the fluid phase alternative pathway regulator Factor H and the membrane regulator Membrane Cofactor Protein MCP (CD46). The functional characterization of the mutant proteins allows to define the pathophysiological events on a molecular level. These new concepts and data on disease mechanisms allowed establishing new diagnostic and promising therapeutic approaches for several human kidney diseases. Molecular biology, clinics and therapy are discussed in this volume.

Inhalt
The complement system in renal diseases.- Complement in renal transplantation.- C1q and the glomerulonephritides: therapeutic approaches for the treatment of complement-mediated kidney diseases.- Complement deficient mice as model systems for kidney diseases.- Non-Shiga toxin-associated hemolytic uremic syndrome.- Role of complement and Factor H in hemolytic uremic syndrome.- Genetic testing in atypical HUS and the role of membrane cofactor protein (MCP; CD46) and Factor I.- Towards a new classification of hemolytic uremic syndrome.- Therapeutic strategies for atypical and recurrent hemolytic uremic syndromes (HUS).- Complement defects in children which result in kidney diseases: diagnosis and therapy.- The role of complement in membranoproliferative glomerulonephritis.- The experience of a patient advocacy group.