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Related Concept Videos

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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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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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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Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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Antimicrobial proteins are important components of the immune system. They aid the body in combating pathogens by either killing them directly or hindering their replication processes. Four main types of antimicrobial substances are interferons, the complement system, iron-binding proteins, and antimicrobial proteins.
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Depletion of Specific Cell Populations by Complement Depletion
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Published on: February 5, 2010

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Complementopathies.

Andrea C Baines1, Robert A Brodsky1

  • 1Division of Hematology, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA.

Blood Reviews
|February 21, 2017
PubMed
Summary
This summary is machine-generated.

The complement system, crucial for immunity, can cause disease when improperly activated. Understanding these "complementopathies" like PNH and aHUS may lead to targeted therapies.

Keywords:
Alternative pathway of complementAtypical hemolytic uremic syndromeCold agglutinin diseaseComplementopathyHELLP syndromeParoxysmal nocturnal hemoglobinuria

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Area of Science:

  • Immunology
  • Hematology
  • Pathophysiology

Background:

  • The complement system is vital for innate immunity, requiring strict regulation.
  • Dysregulation of complement contributes to various human diseases, termed "complementopathies".
  • Deficiencies increase infection risk, while inappropriate activation drives pathology.

Purpose of the Study:

  • To review hematologic disorders driven by complement dysregulation.
  • To focus on paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), and cold agglutinin disease (CAD).
  • To explore the role of complement in the pathophysiology of these diseases.

Main Methods:

  • Literature review of complement system's role in hematologic disorders.
  • Analysis of pathophysiology in PNH, aHUS, and CAD.
  • Synthesis of current understanding of complement-driven diseases.

Main Results:

  • Complement system dysregulation is central to PNH, aHUS, and CAD.
  • These disorders demonstrate the pathological impact of inappropriate complement activation.
  • Understanding complement's role is key to disease mechanisms.

Conclusions:

  • Targeting the complement cascade offers potential therapeutic strategies.
  • Further research into complementopathies can improve patient outcomes.
  • The complement system's dual role in defense and disease is highlighted.