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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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 disorders...
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...
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...
Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

Overview
Cytotoxic T Cells-mediated Immune Response01:27

Cytotoxic T Cells-mediated Immune Response

Cytotoxic T cells are a vital component of the immune system. They have the remarkable ability to identify and target antigens on infected or abnormal cells. These antigens often originate from intracellular pathogens such as viruses or abnormal proteins cancer cells produce.
Immunological surveillance is the ability of immune cells to monitor and eliminate infected cells with intracellular pathogens, neoplastically transformed cells, and cells with non-self antigens. Cytotoxic T cells and NK...
Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

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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Related Experiment Video

Updated: May 13, 2026

Generation of Human Alloantigen-specific T Cells from Peripheral Blood
09:47

Generation of Human Alloantigen-specific T Cells from Peripheral Blood

Published on: November 21, 2014

Partial defects of T-cell development associated with poor T-cell function.

Luigi D Notarangelo1

  • 1Division of Immunology and the Manton Center for Orphan Disease Research, Children's Hospital Boston, Boston, MA 02115, USA. luigi.notarangelo@childrens.harvard.edu

The Journal of Allergy and Clinical Immunology
|March 8, 2013
PubMed
Summary
This summary is machine-generated.

Genetic defects in T-cell development cause severe infections and immune dysregulation. Recent advances identify new gene defects leading to incomplete T-cell issues, impacting immune responses and increasing infection susceptibility.

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Last Updated: May 13, 2026

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09:47

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Published on: November 21, 2014

Isolation of CD4+ T-cells and Analysis of Circulating T-follicular Helper (cTfh) Cell Subsets from Peripheral Blood Using 6-color Flow Cytometry
07:39

Isolation of CD4+ T-cells and Analysis of Circulating T-follicular Helper (cTfh) Cell Subsets from Peripheral Blood Using 6-color Flow Cytometry

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Area of Science:

  • Immunology
  • Genetics
  • Molecular Biology

Background:

  • Severe combined immune deficiency (SCID) diseases are genetic defects causing lack of T cells and early-life infections.
  • Historically, SCID represented the primary model for T-cell development genetic disorders.
  • Recent genomic advances reveal more gene defects causing partial T-cell deficiencies.

Purpose of the Study:

  • To review the evolving understanding of genetic defects in T-cell development and function.
  • To highlight the spectrum of T-cell deficiencies beyond SCID.
  • To characterize the clinical phenotypes associated with these genetic immune disorders.

Main Methods:

  • Review of recent literature on genetic immune deficiencies.
  • Analysis of genome sequencing data for identifying novel gene defects.
  • Clinical phenotyping of patients with T-cell development and function defects.

Main Results:

  • Identification of numerous gene defects causing incomplete T-cell development or function.
  • Association of these defects with recurrent severe infections, particularly cutaneous viral infections.
  • Observation of significant immune dysregulation as a key clinical feature.

Conclusions:

  • Genetic defects in T-cell development encompass a broader spectrum than previously thought.
  • Immune dysregulation and susceptibility to infections are hallmarks of these genetic conditions.
  • Advances in genomics are crucial for diagnosing and understanding these complex immune disorders.