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Related Concept Videos

Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

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 sickness, a systemic...
Hypersensitivities01:30

Hypersensitivities

Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
Humoral Immune Responses01:36

Humoral Immune Responses

Overview
Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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...
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...
Transcytosis of IgG01:15

Transcytosis of IgG

Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...

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Updated: May 10, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

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Published on: December 15, 2011

The hyper IgM syndromes.

Nashmia Qamar1, Ramsay L Fuleihan

  • 1Division of Allergy and Immunology, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Clinical Reviews in Allergy & Immunology
|June 26, 2013
PubMed
Summary
This summary is machine-generated.

Hyper IgM syndromes are rare genetic immune disorders affecting immunoglobulin isotype switching due to CD40 ligand/CD40 pathway defects. Identifying genetic causes allows for definitive diagnosis and targeted therapies like stem cell transplantation or antibody replacement.

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Last Updated: May 10, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

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Published on: December 15, 2011

Analysis of Somatic Hypermutation in the JH4 intron of Germinal Center B cells from Mouse Peyer's Patches
09:35

Analysis of Somatic Hypermutation in the JH4 intron of Germinal Center B cells from Mouse Peyer's Patches

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Simultaneous Detection of Different Antibody Classes in a Multiplexed Serological Test
05:25

Simultaneous Detection of Different Antibody Classes in a Multiplexed Serological Test

Published on: July 14, 2023

Area of Science:

  • Immunology
  • Genetics
  • Molecular Biology

Background:

  • Hyper IgM syndromes are inherited immune deficiencies.
  • Characterized by impaired immunoglobulin isotype switching.
  • Caused by defects in the CD40 ligand/CD40 signaling pathway.

Purpose of the Study:

  • To review the genetic defects causing Hyper IgM syndromes.
  • To delineate the clinical manifestations of different Hyper IgM diseases.
  • To discuss diagnostic and therapeutic strategies.

Main Methods:

  • Genetic analysis of affected individuals.
  • Review of identified genetic defects.
  • Correlation of genotype with clinical phenotype.

Main Results:

  • X-linked forms result from CD40 ligand or NF-κB essential modulator defects.
  • Autosomal recessive forms involve CD40 or downstream signaling defects (AID, UNG, PUM2).
  • CD40 pathway defects impact T cell, B cell, and monocyte functions differently based on the specific gene affected.

Conclusions:

  • Numerous genetic defects in Hyper IgM syndrome are identified, enabling precise diagnosis and genetic screening.
  • Understanding specific genetic causes allows for delineation of distinct clinical manifestations.
  • Therapeutic options include stem cell transplantation for combined immunodeficiencies and antibody replacement for humoral immunodeficiencies.