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Related Concept Videos

Complement System01:27

Complement System

13.2K
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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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.
Interferons
Interferons (IFNs) are proteins produced by lymphocytes, macrophages, and fibroblasts infected with viruses. While IFNs cannot prevent viruses from entering and...
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Complementation Tests00:49

Complementation Tests

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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.
Organisms heterozygous for different mutations are crossed pairwise in all combinations. If present on different genes, the mutations can complement each other by providing the missing...
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Inflammatory Response01:28

Inflammatory Response

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An inflammatory response is a localized, nonspecific immune reaction that occurs when a tissue is injured. It is characterized by redness, swelling, heat, and pain, which are commonly called the cardinal signs and symptoms of inflammation. Inflammation can sometimes result in a loss of function.
Inflammation can be triggered by various stimuli, such as impact, abrasion, chemical irritation, infections, and extreme hot or cold temperatures. These can damage cells and connective tissue fibers,...
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Receptor Downregulation in MVBs01:15

Receptor Downregulation in MVBs

3.0K
Multivesicular bodies (MVBs) are mature endosomes that sort ubiquitinated proteins and then fuse with lysosomes to degrade the sorted proteins. Epidermal growth factor (EGF) and its receptor (EGFR) form a complex that can be internalized through endocytosis, sorted into an MVB, and later degraded.
The EGFR can initiate signaling pathways that  lead to cell proliferation, migration, and differentiation. Overexpression of EGFR  stimulates cells to proliferate. Excessive  EGFR...
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T Cell Types and Functions01:24

T Cell Types and Functions

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When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
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Updated: Apr 19, 2026

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment
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High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

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Complement regulators in human disease: lessons from modern genetics.

M K Liszewski1, J P Atkinson1

  • 1Division of Rheumatology, Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO, USA.

Journal of Internal Medicine
|December 16, 2014
PubMed
Summary
This summary is machine-generated.

The complement system, an ancient immune defense, involves over 60 proteins. Genetic variations can cause inflammatory diseases, leading to new therapies targeting complement system components like C5.

Keywords:
age-related macular degenerationatypical haemolytic uraemic syndromecomplement regulationeculizumabgeneticstherapeutics

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In Vitro Methods for Comparing Target Binding and CDC Induction Between Therapeutic Antibodies: Applications in Biosimilarity Analysis
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Area of Science:

  • Immunology
  • Molecular Biology
  • Genetics

Background:

  • The complement system is a crucial part of the humoral immune system, originating over a billion years ago.
  • It comprises nearly 60 proteins functioning through three activation pathways and a common terminal cytolytic pathway.
  • Initially identified in the 19th century for its role in bacterial lysis, it acts as a complement to antibodies.

Purpose of the Study:

  • To elucidate the structure and function of complement system components using modern molecular biology and genetics.
  • To identify genetic variations, including rare variants and common polymorphisms, that influence complement system function.
  • To understand how these genetic factors predispose individuals to human diseases characterized by 'hyperinflammatory complement phenotypes' (complementopathies).

Main Methods:

  • Utilizing modern molecular biology techniques to study the structure of complement system proteins.
  • Employing genetic analysis to identify variants and polymorphisms in complement regulators, particularly within the alternative pathway.
  • Investigating the functional consequences of these genetic alterations on complement system activity and disease predisposition.

Main Results:

  • Detailed elucidation of the structure of numerous complement system components.
  • Identification of function-altering rare variants and common polymorphisms, especially in regulators of the alternative pathway.
  • Demonstration that these genetic variations can lead to 'hyperinflammatory complement phenotypes', increasing susceptibility to certain diseases.

Conclusions:

  • The complement system's complexity and genetic underpinnings are increasingly understood.
  • Genetic variations in the complement system are significant contributors to human inflammatory diseases.
  • Targeted therapies, such as monoclonal antibodies against C5, are emerging for treating 'complementopathies'.