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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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Complementation Tests00:49

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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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Immunodeficiency Diseases01:25

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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Factors Affecting the Risk of Infection01:26

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The hosts' susceptibility to infection depends on several factors. The integrity of the skin and mucous membranes helps protect the body against microbial attacks. When the skin is altered, the chance of infection, limb loss, and even death increases.
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Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
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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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Inherited defects in the complement system.

Lucia Leonardi1, Francesco La Torre2, Annarosa Soresina3

  • 1Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.

Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology
|January 26, 2022
PubMed
Summary
This summary is machine-generated.

Inherited complement deficiencies disrupt immune responses, leading to severe infections and autoimmune diseases. Early diagnosis and management are crucial for affected individuals, particularly children.

Keywords:
alternativeclassicalcomplementdeficiencyinheritedlectinpathwayssystem

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Area of Science:

  • Immunology
  • Genetics

Background:

  • The complement system is vital for innate and adaptive immunity.
  • Dysregulation of the complement system contributes to infections and autoimmune conditions.
  • Inherited deficiencies affect all complement components and regulators, often manifesting in childhood.

Purpose of the Study:

  • To provide an overview of clinical disorders associated with complement deficiencies.
  • To describe current diagnostic strategies for complement deficiencies.
  • To highlight the importance of comprehensive characterization and management.

Main Methods:

  • Literature review of complement system function and deficiencies.
  • Analysis of clinical manifestations and disease associations.
  • Summary of diagnostic approaches and management guidelines.

Main Results:

  • Complement deficiencies, though rare, can cause severe, early-onset health issues.
  • Three distinct pathways activate the complement system.
  • Diagnostic strategies are essential for identifying and managing these conditions.

Conclusions:

  • Understanding complement system dysregulation is key to managing associated diseases.
  • Comprehensive diagnostic and management plans are critical for patients with complement deficiencies.
  • Further research into complement-related disorders can improve patient outcomes.