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Immunodeficiency Diseases

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.
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T Cell Activation and Clonal Selection

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.
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Special Features of Adaptive Immunity

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Cytotoxic T Cells-mediated Immune Response

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Related Experiment Video

Updated: May 15, 2026

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

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

Published on: January 7, 2019

Functional T cell immunodeficiencies (with T cells present).

Luigi D Notarangelo¹

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

Annual Review of Immunology

|January 10, 2013

Summary

This summary is machine-generated.

Severe combined immunodeficiency (SCID) involves genetic defects that prevent T cell development or function. Understanding intracellular signaling is crucial for maintaining T cell homeostasis and preventing immune dysregulation.

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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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Published on: January 7, 2019

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Published on: December 6, 2014

Area of Science:

Immunology
Molecular Biology
Genetics

Background:

Severe combined immunodeficiency (SCID) is a group of fatal genetic disorders.
Defects can abrogate T cell development or impair T cell function via intracellular signaling pathways.
Functional T cell immunodeficiencies lead to immune dysregulation, infections, and malignancy risk.

Purpose of the Study:

To highlight the critical role of intracellular signaling in T cell homeostasis.
To underscore the importance of studying these rare conditions and animal models.

Main Methods:

Analysis of genetic defects causing SCID and functional T cell immunodeficiencies.
Investigation of intracellular signaling pathways in T cells.
Study of patient cohorts and relevant animal models.

Main Results:

Genetic defects impacting T cell development or function are linked to SCID and related disorders.
Intracellular signaling pathways are essential for maintaining T cell homeostasis.
Dysfunctional signaling contributes to immune dysregulation and increased cancer risk.

Conclusions:

Intracellular signaling is vital for normal T cell function and immune system stability.
Research into these rare immunodeficiencies offers insights into fundamental T cell biology.
Targeting signaling pathways may hold therapeutic potential for immune disorders.