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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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Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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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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Renal Corpuscle01:20

Renal Corpuscle

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The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
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Antibody Actions01:26

Antibody Actions

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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.
Neutralization
Antibodies can bind to pathogens, preventing them from infecting host cells. This process...
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Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

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Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
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Antimicrobial Proteins01:23

Antimicrobial Proteins

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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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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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Complement inhibition in C3 glomerulopathy.

Carla M Nester1, Richard J H Smith1

  • 1Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Divisions of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.

Seminars in Immunology
|July 13, 2016
PubMed
Summary
This summary is machine-generated.

C3 glomerulopathy (C3G) is a kidney disease characterized by C3 protein deposits. Understanding complement dysregulation is key to developing targeted treatments like complement inhibition.

Keywords:
C3 glomerulonephritisC3 glomerulopathyComplementDense deposit diseaseEculizumabMembranoproliferative glomerulonephritis

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Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • C3 glomerulopathy (C3G) is a group of kidney diseases defined by specific renal biopsy findings.
  • These findings include predominant C3 complement protein deposition, identifiable via immunofluorescence and electron microscopy.
  • Complement system dysregulation is the established cause of C3G, driven by genetic and acquired factors.

Purpose of the Study:

  • To provide a comprehensive overview of C3 glomerulopathy (C3G).
  • To discuss the definition, pathology, and underlying causes of C3G, focusing on complement dysregulation.
  • To review emerging molecular markers and the potential of complement inhibition as a therapeutic strategy.

Main Methods:

  • Review of existing literature on C3 glomerulopathy.
  • Analysis of pathological features from renal biopsies (immunofluorescence, electron microscopy).
  • Discussion of genetic and acquired factors contributing to complement dysregulation.

Main Results:

  • C3G is characterized by specific C3 deposition patterns in the glomeruli.
  • Complement dysregulation, involving genetic and acquired factors, is causally linked to C3G.
  • Biomarkers are enhancing the understanding of C3G pathology.

Conclusions:

  • C3G encompasses a spectrum of kidney diseases unified by C3 deposition and complement dysregulation.
  • Advances in understanding molecular markers and complement pathways are crucial for C3G.
  • Complement inhibition shows promise as a targeted treatment for C3G.