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Related Concept Videos

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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T Cell Activation and Clonal Selection01:22

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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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T Cell Types and Functions01:24

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When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
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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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Diversity of Antigen Receptors01:28

Diversity of Antigen Receptors

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Antigen receptors are essential components of the immune system crucial in defending the body against foreign invaders. These receptors are present on the surface of B and T cells, enabling them to recognize antigens and mount an appropriate immune response.
Before encountering any antigen, lymphocytes express these receptors. On B cells, the antigen receptor is a membrane-bound antibody molecule called BCR; on T cells, it is a T cell receptor or TCR. B and T cell receptors are composed of two...
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Antigens Involved in Adaptive Immunity01:26

Antigens Involved in Adaptive Immunity

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An antigen is any substance the immune system identifies as foreign and potentially harmful to the body, prompting an immune response. Antigens have two functional properties: immunogenicity and reactivity. Immunogenicity is the ability of an antigen to stimulate a specific immune response. At the same time, reactivity describes the antigen's ability to react with the cells and antibodies produced in response to it.
Complete Antigens
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Evaluation of T Follicular Helper Cells and Germinal Center Response During Influenza A Virus Infection in Mice
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B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency.

Saba Fekrvand1, Shaghayegh Khanmohammadi1, Hassan Abolhassani1,2,3

  • 1Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran.

Frontiers in Immunology
|July 5, 2022
PubMed
Summary

Common variable immunodeficiency (CVID) involves defects in immunoglobulin production. This review examines how altered B- and T-cell differentiation in monogenic CVID contributes to disease.

Keywords:
B cell subsetsCVIDT cell subsetscommon variable immunodeficiency disorderinborn errors of immunitymonogenic disordersprimary immunodeficiency

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low immunoglobulin levels and diverse clinical issues.
  • While genetic causes are identified in 20-50% of CVID cases, the specific impacts on lymphocyte differentiation remain incompletely understood.
  • Existing literature lacks comprehensive reviews on lymphocyte subset alterations in monogenic CVID.

Purpose of the Study:

  • To review the role of aberrant B- and T-cell differentiation in the pathogenesis of CVID.
  • To consolidate current knowledge on lymphocyte abnormalities in patients with monogenic CVID.
  • To highlight the need for further research into the immunogenetic underpinnings of CVID.

Main Methods:

  • Systematic literature review focusing on monogenic defects in CVID.
  • Analysis of studies reporting B-cell and T-cell subset abnormalities in CVID patients.
  • Synthesis of data on the relationship between genetic defects and lymphocyte differentiation pathways.

Main Results:

  • Monogenic defects in CVID can disrupt B-cell development and signaling pathways.
  • Altered B-cell differentiation can impact T-cell populations and overall immune function.
  • Specific genetic defects correlate with distinct patterns of lymphocyte subset abrogation.

Conclusions:

  • Altered B- and T-cell differentiation are significant contributors to CVID pathogenesis in genetically defined cases.
  • Understanding these cellular defects is crucial for diagnosing and managing CVID.
  • Further investigation into the immunogenetics of CVID is warranted to elucidate complex pathogenic mechanisms.