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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...
T Cell Types and Functions01:24

T Cell Types and Functions

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...
Diversity of Antigen Receptors01:28

Diversity of Antigen Receptors

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...
T Cell Activation and Clonal Selection01:22

T Cell Activation and Clonal Selection

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...
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...
Defense Against Bacterial Pathogens01:31

Defense Against Bacterial Pathogens

The human immune system is a complex network of cells, tissues, and organs that work together to defend the body against bacterial infections. It consists of various immune cells, each playing a specific role in the defense mechanism.
Phagocytes
Phagocytes are the frontline soldiers of the immune system. They include neutrophils and macrophages. Neutrophils are the most abundant type of white blood cell and are quickly mobilized to the site of infection. Macrophages are larger cells that patrol...

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Evaluation of T Follicular Helper Cells and Germinal Center Response During Influenza A Virus Infection in Mice
07:07

Evaluation of T Follicular Helper Cells and Germinal Center Response During Influenza A Virus Infection in Mice

Published on: June 27, 2020

Toll-like receptor function in primary B cell defects.

Thomas U Marron1, Joyce E Yu, Charlotte Cunningham-Rundles

  • 1Mount Sinai School of Medicine, New York, New York 10029, USA.

Frontiers in Bioscience (Elite Edition)
|December 29, 2011
PubMed
Summary
This summary is machine-generated.

Primary immunodeficiency diseases involve genetic defects affecting adaptive and innate immunity. This review explores Toll-like receptor (TLR) defects in B cell disorders like CVID and XLA, highlighting altered TLR signaling.

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Area of Science:

  • Immunology
  • Genetics
  • Molecular Biology

Background:

  • Primary immunodeficiency diseases (PIDs) encompass over 150 genetic defects impacting adaptive and innate immunity.
  • Early PIDs recognized involved B cell defects (hypogammaglobulinemia, poor antibody production).
  • Recent discoveries highlight innate immunity defects, including Toll-like receptor (TLR) pathway dysfunctions.

Purpose of the Study:

  • To review Toll-like receptor (TLR) defects in primary immunodeficiency diseases affecting B cell development.
  • To discuss how B cell genetic defects influence TLR signaling pathways.
  • To explore the interplay between innate and adaptive immunity in PIDs.

Main Methods:

  • Review of existing literature on TLRs and primary immunodeficiency diseases.
  • Analysis of B cell function and TLR responsiveness in patients with CVID and XLA.
  • Discussion of molecular mechanisms linking B cell gene defects to TLR signaling.

Main Results:

  • B cells express TLRs that, upon activation, promote B cell development, antibody production, and cytokine secretion.
  • TLR activation of antigen-presenting cells enhances B cell maturation.
  • Patients with Common Variable Immunodeficiency (CVID) and X-linked Agammaglobulinemia (XLA) exhibit altered TLR responses.

Conclusions:

  • TLR signaling plays a crucial role in B cell immunity and development.
  • Defects in B cell genes can significantly modulate TLR signaling pathways.
  • Understanding TLR defects offers insights into the pathogenesis of PIDs and potential therapeutic targets.