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Related Concept Videos

Primary Lymphoid Organs01:16

Primary Lymphoid Organs

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Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
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Immunodeficiency Diseases01:25

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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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Regulation of Hematopoietic Stem Cells01:01

Regulation of Hematopoietic Stem Cells

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All blood and immune cells are produced from the multipotent hematopoietic stem cells (HSCs) by the process of hematopoiesis. However, they all have a limited life span. In addition, many are depleted in immune surveillance or combatting an injury or infection. This makes blood one of the most regenerative tissues. Hematopoiesis helps replenish these blood and immune cells, restoring the body's normal functioning. However, overproduction of blood and immune cells can make them cancerous or...
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Cell-mediated Immune Responses01:40

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Overview
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Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

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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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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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Updated: Nov 28, 2025

Characterization of Thymic Settling Progenitors in the Mouse Embryo Using In Vivo and In Vitro Assays
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Inborn errors of thymic stromal cell development and function.

Alexandra Y Kreins1,2, Stefano Maio3, Fatima Dhalla4,5

  • 1UCL Great Ormond Street Institute of Child Health, London, UK.

Seminars in Immunopathology
|December 1, 2020
PubMed
Summary
This summary is machine-generated.

Inborn errors of thymic stromal cells can cause severe congenital immunodeficiency and autoimmune disease. This review details their causes, diagnosis, and treatment, including thymus transplantation.

Keywords:
DiGeorge syndromeFOXN1ImmunodeficiencyThymic stromal cellsThymusThymus transplantation

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Area of Science:

  • Immunology
  • Developmental Biology
  • Genetics

Background:

  • The thymus is crucial for T cell development and self-tolerance, relying on specialized stromal microenvironments.
  • Thymic stromal cells, originating from the pharyngeal apparatus, are essential for thymopoiesis and central tolerance.
  • Inborn errors affecting thymic stromal cells lead to severe immune disorders, including T cell immunodeficiency and autoimmunity.

Purpose of the Study:

  • To review the molecular and environmental causes of human thymic stromal cell defects.
  • To emphasize conditions leading to thymic hypoplasia or aplasia and severe congenital immunodeficiency.
  • To discuss diagnostic approaches and management strategies, including curative treatments.

Main Methods:

  • Review of existing literature on thymic stromal cell biology and genetic defects.
  • Analysis of clinical consequences of identified genetic and environmental aetiologies.
  • Evaluation of current and emerging diagnostic and therapeutic strategies.

Main Results:

  • Identified diverse molecular and environmental factors causing thymic stromal cell defects.
  • Highlighted the link between specific defects and thymic hypoplasia/aplasia, leading to severe congenital immunodeficiency.
  • Discussed the potential of novel diagnostic tools and thymus transplantation for treatment.

Conclusions:

  • Inborn errors of thymic stromal cells are a critical cause of life-threatening immune deficiencies.
  • Accurate diagnosis and timely management, potentially including thymus transplantation, are vital.
  • Further research into thymic development and stromal cell function can improve patient outcomes.