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Complement System01:27

Complement System

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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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Antibodies, or immunoglobulins, are critical players in the immune system's arsenal against invading pathogens. Produced by B cells and plasma cells, their primary role is to detect and bind to specific antigens, molecules found on the surface of pathogens like bacteria or viruses. Beyond antigen recognition, antibodies perform several vital functions that contribute to immune defense.
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Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
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Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells

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The complement system and human autoimmune diseases.

Samantha L Coss1, Danlei Zhou1, Gilbert T Chua2

  • 1Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, USA; Department of Pediatrics, The Ohio State University, Columbus, OH, USA.

Journal of Autoimmunity
|December 19, 2022
PubMed
Summary
This summary is machine-generated.

Genetic deficiencies in early complement pathway components are linked to autoimmune diseases like systemic lupus erythematosus (SLE). While rare, specific deficiencies like C4A and acquired C1q deficiency are common in SLE patients, highlighting complement

Keywords:
Antiphospholipid syndromeAutoantibodiesClassical pathwayComplementGene copy number variationsGenetic and acquired deficienciesIdiopathic inflammatory myopathiesJuvenile dermatomyositisPolymorphismsSystemic lupus erythematosusjuvenile idiopathic arthritisrheumatoid arthritistype I interferon induced gene expression

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Area of Science:

  • Immunology
  • Genetics
  • Autoimmunity

Background:

  • Genetic deficiencies in early classical complement pathway components (C1q, C1r, C1s, C4) are strongly associated with systemic lupus erythematosus (SLE).
  • While rare, specific complement deficiencies such as C4A isotype deficiency and acquired C1q deficiency via autoantibodies are frequently observed in SLE patients.
  • Complement system plays a crucial role in immune regulation and the development of autoimmune diseases.

Purpose of the Study:

  • To review the genetic basis of complement deficiencies in autoimmune diseases.
  • To discuss the association between complement C4 genetic diversity and autoimmune conditions.
  • To provide clinical guidance for suspecting and testing complement deficiencies and to outline mechanisms linking these deficiencies to autoimmunity.

Main Methods:

  • Literature review focusing on genetic complement deficiencies and autoimmune diseases.
  • Analysis of existing data on complement C4 genetic diversity and its autoimmune associations.
  • Synthesis of current understanding regarding mechanisms of complement deficiency-induced autoimmunity.

Main Results:

  • Early classical complement pathway deficiencies are potent monogenic causes of SLE, though rare.
  • C4A isotype deficiency and acquired C1q deficiency are prevalent in SLE patients.
  • Complement deficiencies are implicated in the pathogenesis of SLE, inflammatory arthritis, and myositis.

Conclusions:

  • Complement deficiencies, particularly C4A and C1q, are significant factors in SLE development and progression.
  • Understanding complement C4 genetic diversity is crucial for assessing autoimmune disease risk.
  • Clinical suspicion and testing for complement deficiencies are warranted in specific autoimmune contexts to elucidate pathogenic mechanisms.