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Isolation of Glomeruli and In Vivo Labeling of Glomerular Cell Surface Proteins
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Isolation of Glomeruli and In Vivo Labeling of Glomerular Cell Surface Proteins

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Recent insights into C3 glomerulopathy

Thomas D Barbour1, Matthew C Pickering, H Terence Cook

  • 1Centre for Complement & Inflammation Research (CCIR), Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London W12 0NN, UK.

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association
|March 13, 2013
PubMed

Insights

C3 glomerulopathy, a group of rare kidney diseases, involves isolated C3 deposits in the glomerulus due to complement alternative pathway dysregulation. Research highlights genetic factors and ongoing therapeutic evaluations for these conditions.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • C3 glomerulopathy is a classification of rare glomerulonephritis characterized by isolated C3 deposits.
  • Dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and CFHR5 nephropathy are key types within this classification.
  • A common etiology involves dysregulation of the complement alternative pathway (AP).

Purpose of the Study:

  • To review the clinical and histological features of C3 glomerulopathy.
  • To relate these features to underlying molecular mechanisms, emphasizing uncontrolled C3 activation.
  • To discuss gene testing and therapeutic strategies for C3 glomerulopathy.

Main Methods:

  • Review of clinical and histological findings in C3 glomerulopathy.
  • Analysis of molecular mechanisms, including complement alternative pathway dysregulation.
  • Evaluation of genetic testing methods and ongoing therapeutic trials.

Main Results:

  • Isolated C3 deposits in the glomerulus are a hallmark histological feature.
  • Genetic defects and autoantibodies are identified in a proportion of patients.
  • Uncontrolled C3 activation plays a significant role in pathogenesis.

Conclusions:

  • C3 glomerulopathy arises from dysregulated complement alternative pathway activation.
  • Gene testing aids in assessing individuals and families.
  • Effective therapies are under investigation, but knowledge gaps remain regarding natural history and treatment protocols.

Keywords:
complementdense depositeculizumabglomerulonephritiskidney

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