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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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Complement 9 in amyloid deposits.

Annelie Lux1, Juliane Gottwald1, Hans-Michael Behrens1

  • 1Department of Pathology, Christian-Albrechts-University Kiel, Kiel, Germany.

Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis
|June 1, 2021
PubMed
Summary

Complement 9 (C9) is consistently found in amyloid deposits across various types, suggesting a role in the complement cascade activation in amyloidosis. This finding supports the hypothesis linking amyloidosis to complement activation and cell death.

Keywords:
Amyloidamyloidosiscell deathcomplement 9proteomics

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Area of Science:

  • Pathology
  • Immunology
  • Biochemistry

Background:

  • Amyloidosis involves pathological peptide aggregation in tissues.
  • Amyloid deposits contain fibrillar and non-fibrillar components.
  • Complement 9 (C9) was incidentally observed in routine amyloid diagnostics.

Purpose of the Study:

  • To systematically investigate the occurrence and distribution of C9 in diverse amyloid deposits.
  • To compare C9 expression with control proteins like apolipoprotein E (apoE), caspase 3, and complement 3 (C3).
  • To evaluate the potential role of complement activation in amyloidosis.

Main Methods:

  • Immunohistochemical analysis of 118 formalin-fixed, paraffin-embedded tissue samples from the Amyloid Registry Kiel.
  • Assessment of C9, apoE, caspase 3, and C3 expression in relation to eight amyloid types and 18 tissue types.
  • Literature search for proteomic data on amyloid deposit composition.

Main Results:

  • C9 was detected in 99.2% of amyloid deposits, with homogeneous staining in 75.4%.
  • Apolipoprotein E (apoE) was found in 94.9% of cases, with homogeneous staining in 61.9%.
  • Caspase 3 (19.3%) and C3 (36%) were less frequent and showed limited staining.

Conclusions:

  • Complement 9 (C9) is ubiquitously and specifically enriched in amyloid deposits, regardless of amyloid or tissue type.
  • Findings support the hypothesis that amyloidosis activates the complement cascade.
  • Complement activation may lead to membrane attack complex formation and cell death in amyloidosis.