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Related Concept Videos

Humoral Immune Responses01:36

Humoral Immune Responses

Overview
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...
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...
Affinity and Avidity01:41

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Antibody Structure01:10

Antibody Structure

Overview
Antibodies, also known as immunoglobulins (Ig), are essential players of the adaptive immune system. These antigen-binding proteins are produced by B cells and make up 20 percent of the total blood plasma by weight. In mammals, antibodies fall into five different classes, which each elicits a different biological response upon antigen binding.
The Y-Shaped Structure of Antibodies Consists of Four Polypeptide Chains
Antibodies consist of four polypeptide chains: two identical heavy...
Antibody Actions01:26

Antibody Actions

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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Updated: May 10, 2026

Detection and Enrichment of Rare Antigen-specific B Cells for Analysis of Phenotype and Function
09:25

Detection and Enrichment of Rare Antigen-specific B Cells for Analysis of Phenotype and Function

Published on: February 16, 2017

Primary antibody deficiencies.

Anne Durandy1, Sven Kracker, Alain Fischer

  • 1National Institute of Health and Medical Research, INSERM U768, Necker Children's Hospital, F-75015 Paris, France. anne.durandy@inserm.fr

Nature Reviews. Immunology
|June 15, 2013
PubMed
Summary
This summary is machine-generated.

Primary antibody deficiencies (PADs) are common inherited immune disorders. Research using advanced genetic techniques identifies new genes and pathways in B cell development, impacting infections, autoimmunity, and cancer risks.

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Last Updated: May 10, 2026

Detection and Enrichment of Rare Antigen-specific B Cells for Analysis of Phenotype and Function
09:25

Detection and Enrichment of Rare Antigen-specific B Cells for Analysis of Phenotype and Function

Published on: February 16, 2017

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
06:29

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells

Published on: January 29, 2014

Generation of Discriminative Human Monoclonal Antibodies from Rare Antigen-specific B Cells Circulating in Blood
13:14

Generation of Discriminative Human Monoclonal Antibodies from Rare Antigen-specific B Cells Circulating in Blood

Published on: February 6, 2018

Area of Science:

  • Immunology
  • Genetics
  • Molecular Biology

Background:

  • Primary antibody deficiencies (PADs) represent the most frequent inherited immunodeficiencies.
  • Understanding the genetic basis and molecular pathways of PADs is crucial for diagnosis and treatment.

Purpose of the Study:

  • To review current knowledge on primary antibody deficiencies.
  • To elucidate the mechanisms underlying B cell development and function in PADs.
  • To correlate genetic defects with clinical manifestations.

Main Methods:

  • Review of recent literature on PADs.
  • Analysis of data from whole-exome sequencing studies.
  • Examination of findings from mouse genetic engineering models.

Main Results:

  • Novel genes involved in PAD pathogenesis have been identified.
  • Molecular pathways regulating B cell development and function are increasingly characterized.
  • PADs are categorized by B cell intrinsic, B cell extrinsic, or undefined mechanisms.

Conclusions:

  • Advances in genetic technologies are revolutionizing the study of PADs.
  • Understanding PAD mechanisms provides insight into B cell biology.
  • Clinical manifestations including infections, autoimmunity, and cancer are linked to specific PADs.