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Defective complement inhibitory function predisposes to renal disease

Anuja Java1, John Atkinson, Jane Salmon

  • 1Department of Medicine, Washington University School of Medicine, St. Louis, Missouri 63110, USA. AJAVA@dom.wustl.edu

Annual Review of Medicine
|November 6, 2012
PubMed

Insights

The complement system significantly impacts human kidney diseases, with genetic mutations and excessive activation leading to conditions like atypical hemolytic uremic syndrome. Understanding these complement pathways enables targeted therapies for renal diseases.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • The complement system's role in renal disease is established in immune-complex disorders and dense deposit disease.
  • Recent research highlights complement regulatory gene mutations in thrombotic microangiopathies and preeclampsia.

Purpose of the Study:

  • To review the involvement of complement system dysregulation in various human renal diseases.
  • To discuss the link between genetic mutations, complement overactivation, and thrombotic microangiopathies.

Main Methods:

  • Literature review of complement system's role in renal pathology.
  • Analysis of genetic mutations in complement regulatory genes.
  • Examination of the procoagulant state induced by complement activation.

Main Results:

  • Mutations in complement regulatory genes predispose individuals to thrombotic microangiopathies (e.g., atypical hemolytic uremic syndrome, C3 and C1q glomerulopathies).
  • Excessive complement activation on endothelial cells, caused by autoantibodies or protein deficiencies, promotes a procoagulant state.
  • These mechanisms are implicated in systemic lupus erythematosus, dense deposit disease, preeclampsia, and antiphospholipid syndrome.

Conclusions:

  • Genetic alterations in complement regulatory proteins are key drivers of specific renal diseases.
  • Targeted therapies blocking complement activation have emerged based on understanding these genetic and functional consequences.

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