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Updated: Feb 13, 2026

Measuring the 50% Haemolytic Complement CH50 Activity of Serum
Published on: March 29, 2010
Evolving complexity of complement-related diseases: C3 glomerulopathy and atypical haemolytic uremic syndrome
1Department of Medicine, Imperial College London, London, UK.
Insights
Recent advances clarify the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS), revealing complement alternative pathway dysregulation. New insights suggest therapeutic targets for these kidney diseases.
Area of Science:
- Nephrology
- Immunology
- Complement System Biology
Background:
- C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS) are kidney diseases linked to complement alternative pathway dysregulation.
- Understanding their pathogenesis is crucial for developing effective treatments.
Purpose of the Study:
- To review recent advances in the pathology of C3 glomerulopathy and aHUS.
- To explore new insights into classification and pathogenesis.
- To highlight emerging therapeutic strategies targeting the complement cascade.
Main Methods:
- Literature review of recent articles on C3 glomerulopathy and aHUS.
- Analysis of new findings on complement pathway abnormalities.
- Synthesis of evidence regarding monoclonal gammopathy and treatment.
Main Results:
- New insights into C3 glomerulopathy classification and its relationship with immune complex-mediated glomerulonephritis.
- Evidence for overlap in pathogenesis between C3 glomerulopathy and aHUS.
- Growing evidence linking monoclonal gammopathy to C3 glomerulopathy/aHUS in older patients, with potential for treatment of the plasma cell clone to improve kidney outcomes.
Conclusions:
- Recent research has significantly advanced the understanding of C3 glomerulopathy and aHUS.
- Complement dysregulation is a key factor, with novel therapeutic agents emerging.
- Monoclonal gammopathy represents an important treatable cause in specific patient populations.
Purpose Of Review:
The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS).
Recent Findings:
C3 glomerulopathy and aHUS are associated with abnormalities of control of the alternative pathway of complement. Recent articles have provided new insights into the classification of C3 glomerulopathy and its relationship to idiopathic immune complex-mediated glomerulonephritis. They suggest that there may be considerable overlap in pathogenesis between these entities and have indicated novel ways in which classification may be improved. There is increasing evidence that monoclonal gammopathy may cause C3 glomerulopathy or aHUS in older patients and emerging evidence that treatment of the underlying plasma cell clone may ameliorate the kidney disease.
Summary:
Recent work has provided new insights into the causes of C3 glomerulopathy and aHUS, and the mechanism by which complement is dysregulated. This is of particular importance with the advent of new therapeutic agents which can specifically target different parts of the complement cascade.
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