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Related Concept Videos

Complement System01:27

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The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
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Complementation Tests00:49

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A complementation test is a simple cross to identify whether the two mutations are located on the same gene or different genes. It was first performed by Edward Lewis in the 1940s while working on fruit flies. He developed the test to identify the location and arrangement of different mutations on chromosomes.
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Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
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Functional Hemolytic Test for Complement Alternative Pathway Convertase Activity.

Marloes A H M Michels1, Nicole C A J van de Kar1, Elena B Volokhina1,2

  • 1Department of Pediatric Nephrology, Radboud Institute for Molecular Life Sciences, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.

Methods in Molecular Biology (Clifton, N.J.)
|April 13, 2021
PubMed
Summary

The complement system, vital for immunity, can cause kidney damage when dysregulated. This study presents a method to measure alternative pathway convertase activity, detecting factors causing overactivity in kidney diseases.

Keywords:
Alternative pathwayAtypical hemolytic uremic syndromeC3 glomerulopathyC3 nephritic factorComplementConvertaseEculizumabHemolytic assayMutationRabbit erythrocytes

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Area of Science:

  • Immunology
  • Nephrology
  • Molecular Biology

Background:

  • The complement system is crucial for innate immunity but can cause host cell damage, particularly in kidney glomeruli, when dysregulated.
  • Alternative pathway convertases are central to complement activation, normally under strict regulation.
  • Dysregulation of the alternative pathway is implicated in kidney diseases like C3 glomerulopathy and atypical hemolytic uremic syndrome.

Purpose of the Study:

  • To describe a method for measuring the activity and stability of alternative pathway convertases.
  • To detect aberrant serum factors that lead to complement convertase overactivity.
  • To aid in the diagnosis of complement-mediated kidney diseases.

Main Methods:

  • Quantification of alternative pathway convertase activity.
  • Assessment of convertase complex stability.
  • Detection of autoantibodies (C3 nephritic factors) and genetic variants influencing convertase function.

Main Results:

  • The described method can successfully measure complement convertase activity and stability.
  • Aberrant serum factors, including C3 nephritic factors and genetic variants, were identified as causes of convertase overactivity.
  • The findings highlight the role of these factors in the pathogenesis of complement-mediated kidney diseases.

Conclusions:

  • Measuring alternative pathway convertase activity and stability is essential for diagnosing complement dysregulation.
  • This method can identify specific factors contributing to overactivity, aiding in disease understanding.
  • The findings support the development of targeted therapies for complement-mediated kidney disorders.