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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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.
There are three main causes of immunodeficiency disorders...
Humoral Immune Responses01:36

Humoral Immune Responses

Overview
What is the Immune System?01:38

What is the Immune System?

Overview
Development of Immunocompetence01:22

Development of Immunocompetence

The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
Respiratory Syncytial Virus Disease01:29

Respiratory Syncytial Virus Disease

Human respiratory syncytial virus (RSV) is a widespread pathogen that primarily targets infants and young children but also poses a serious health risk to elderly and immunocompromised individuals. Belonging to the Pneumoviridae family, RSV is a negative-sense, single-stranded RNA virus within the Pneumovirus genus. Its global health burden is significant, with millions of cases annually resulting in hospitalizations and mortality, particularly in resource-limited settings. Although most...
Primary Lymphoid Organs01:16

Primary Lymphoid Organs

Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...

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Related Experiment Video

Updated: Jun 10, 2026

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR
14:14

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Published on: December 6, 2014

Primary immunodeficiency syndromes.

Mary A Slatter1, Andrew R Gennery

  • 1Newcastle General Hospital, Westgate Road, Newcastle upon Tyne, NE4 6BE, England.

Advances in Experimental Medicine and Biology
|August 7, 2010
PubMed
Summary
This summary is machine-generated.

DNA repair pathways are crucial for genomic integrity and adaptive immunity. Defects in these pathways lead to immunodeficiency and radiosensitivity, impacting VDJ recombination and antibody diversity.

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Area of Science:

  • Molecular Biology
  • Immunology
  • Genetics

Background:

  • DNA repair pathways maintain genomic stability against exogenous and endogenous damage.
  • Defects in DNA repair can cause mutations, cancer, and cell death.
  • Adaptive immunity relies on DNA repair mechanisms for generating diverse lymphocyte receptors.

Purpose of the Study:

  • To elucidate the mechanisms linking DNA repair to adaptive immunity development.
  • To describe the clinical consequences of DNA repair defects in primary immunodeficiency.
  • To review treatment advancements for these conditions.

Main Methods:

  • Review of molecular mechanisms of DNA repair in V(D)J recombination, class switch recombination, and somatic hypermutation.
  • Analysis of clinical data from patients with primary immunodeficiency due to DNA repair defects.
  • Synthesis of recent findings on newly identified genes involved in human disease.

Main Results:

  • DNA repair proteins are essential for VDJ recombination, antibody class switching, and somatic hypermutation.
  • Deficiencies in DNA repair are linked to primary immunodeficiency and radiosensitivity.
  • Identification of novel genes implicated in human diseases associated with DNA repair defects.

Conclusions:

  • DNA repair pathways are integral to adaptive immune system development and function.
  • Understanding these pathways offers insights into primary immunodeficiencies and potential therapeutic targets.
  • Continued research into DNA repair mechanisms is vital for advancing treatments for immunodeficiency and related disorders.