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Related Concept Videos

B Cell Activation and Differentiation01:24

B Cell Activation and Differentiation

The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
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...
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...
Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...

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Related Experiment Video

Updated: Jul 10, 2026

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

Published on: April 20, 2021

Deciphering B cell Maturation Dynamics in Hyper-IgM Syndromes.

Hande Üçler Çınar1, Murat Cansever2, Şerife Erdem3,4,5

  • 1Division of Pediatric Allergy and Immunology, Kayseri City Hospital, Kayseri, Türkiye. uclerhande@gmail.com.

Journal of Clinical Immunology
|July 9, 2026
PubMed
Summary
This summary is machine-generated.

Hyper-IgM syndromes (HIGM) involve defective B cell class-switch recombination (CSR). This study distinguishes B cell defects in CD40L deficiency versus AICDA mutations, aiding diagnosis and monitoring.

Keywords:
AICDAB cell maturationCD40LClass-switch recombinationHyper-IgM syndrome

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Area of Science:

  • Immunology
  • Primary Immunodeficiency Diseases
  • B cell Biology

Background:

  • Hyper-IgM syndromes (HIGM) are primary immunodeficiencies characterized by defective class-switch recombination (CSR) and impaired humoral immunity.
  • Genetic causes like CD40L and AICDA mutations are known, but B cell maturation dynamics across HIGM subtypes require detailed comparison.

Purpose of the Study:

  • To comprehensively characterize B cell immunophenotypes and functional responses in patients with HIGM.
  • To delineate mutation-specific differences in B cell maturation and proliferation in HIGM subtypes.

Main Methods:

  • Multiparameter flow cytometry was used to analyze peripheral blood mononuclear cells from four HIGM patients (one CD40L, three AICDA mutations) and healthy controls.
  • B cell subsets were defined using CD19, CD20, CD24, CD27, CD38, IgD, and IgM expression.
  • B cell proliferation was assessed post-CpG stimulation.

Main Results:

  • All patients showed reduced class-switched memory B cells and increased naive B cells, indicating defective CSR.
  • CD40L deficiency led to plasmablast depletion and germinal center formation failure.
  • AICDA mutations showed preserved CD27 expression but variable transitional and plasmablast populations, suggesting intrinsic CSR failure despite intact T cell-dependent activation; proliferation responses were heterogeneous.

Conclusions:

  • Detailed immunophenotyping reveals distinct B cell maturation arrest points in CD40L- versus AICDA-associated HIGM.
  • Flow cytometry analysis of B cell subsets offers valuable insights into HIGM disease mechanisms.
  • Findings support differential diagnosis, clinical monitoring, and therapeutic decision-making in HIGM.