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Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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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.
There are three main causes of immunodeficiency...
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B Cell Activation and Differentiation01:24

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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...
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T Cell Activation and Clonal Selection01:22

T Cell Activation and Clonal Selection

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T cells are integral to our adaptive immune system, recognizing and effectively responding to foreign antigens. T cell activation and clonal selection are pivotal in orchestrating this immune response. This article elucidates these mechanisms, detailing the roles of cluster of differentiation (CD) markers, major histocompatibility complex (MHC) molecules, costimulatory signals, and the process of clonal selection.
Naive T cells that have not yet encountered an antigen express two primary CD...
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Cells of the Adaptive Immune Response01:23

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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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Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

2.6K
The adaptive immune system, a crucial component of the overall immune response, offers a highly specialized defense against pathogens. It involves specific cell types and features, enabling it to combat infections effectively and efficiently.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
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Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

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Related Experiment Video

Updated: Dec 8, 2025

In Vitro Differentiation Model of Human Normal Memory B Cells to Long-lived Plasma Cells
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In Vitro Differentiation Model of Human Normal Memory B Cells to Long-lived Plasma Cells

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Defective Bcl-2 expression in memory B cells from common variable immunodeficiency patients.

L Del Pino Molina1, J M Torres Canizales1, O Pernía2

  • 1Clinical Immunology Department, La Paz University Hospital, Lymphocyte Pathophysiology in Immunodeficiencies Group La Paz Institute for Health Research (IdiPAZ) and Center for Biomedical Network Research on Rare Diseases (CIBERER U767), Madrid, Spain.

Clinical and Experimental Immunology
|September 22, 2020
PubMed
Summary

Common variable immunodeficiency (CVID) patients show reduced Bcl-2 protein in memory B cells, impacting survival. Defects in NF-κB signaling impair BCL-XL mRNA production in naive B cells, contributing to apoptosis.

Keywords:
B cellsanti-apoptotic proteinscommon variable immunodeficiency

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Area of Science:

  • Immunology
  • Molecular Biology

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency marked by low antibody levels and altered B cell populations.
  • B cell differentiation and survival depend on intracellular signaling pathways, including NF-κB activation, which regulates pro-survival factors like Bcl-2.

Purpose of the Study:

  • To investigate if defects in intracellular signaling pathways contribute to impaired B cell differentiation, survival, and activation in CVID patients.
  • To examine the role of NF-κB-regulated signaling in B cell apoptosis in CVID.

Main Methods:

  • Analysis of Bcl-2 family protein expression in memory B cells from CVID patients.
  • Assessment of anti-apoptotic mRNA levels (BCL-XL, AICDA) in naive B cells after in vitro activation with CD40L and IL-21.

Main Results:

  • CVID patients exhibited reduced Bcl-2 protein levels in memory B cells.
  • Naive B cells from CVID patients showed decreased BCL-XL and AICDA mRNA levels upon activation.
  • These findings suggest impaired prosurvival signaling in B cells from CVID patients.

Conclusions:

  • Reduced Bcl-2 in memory B cells may compromise their long-term survival in CVID.
  • Potential defects in NF-κB activity in naive B cells could hinder BCL-XL induction, promoting B cell apoptosis and impacting germinal center function.