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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.
There are three main causes of immunodeficiency disorders...

Cytomegalovirus Disease

Cytomegalovirus Disease

Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...

Development of Immunocompetence

Development of Immunocompetence

The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...

Special Features of Adaptive Immunity

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,...

Cell-mediated Immune Responses

Cell-mediated Immune Responses

Antigens Involved in Adaptive Immunity

Antigens Involved in Adaptive Immunity

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
Complete antigens possess both immunogenicity and reactivity.

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Related Experiment Video

Updated: Jul 7, 2026

Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens

Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens

Published on: September 20, 2024

Common variable immunodeficiency.

Naomi Takahashi¹, Tomohiro Morio

¹Department of Pediatrics and Developmental Biology Tokyo Medical and Dental University, Graduate School.

Nihon Rinsho Men'Eki Gakkai Kaishi = Japanese Journal of Clinical Immunology

|March 4, 2008

Summary

Common variable immunodeficiency (CVID) involves low immunoglobulin levels, leading to infections, autoimmunity, and malignancy. Genetic defects in ICOS, TACI, BAFF-R, and CD19 disrupt B cell development, though exact mechanisms remain unclear.

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Published on: July 9, 2014

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Last Updated: Jul 7, 2026

Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens

Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens

Published on: September 20, 2024

Development of an IFN-γ ELISpot Assay to Assess Varicella-Zoster Virus-specific Cell-mediated Immunity Following Umbilical Cord Blood Transplantation

Development of an IFN-γ ELISpot Assay to Assess Varicella-Zoster Virus-specific Cell-mediated Immunity Following Umbilical Cord Blood Transplantation

Published on: July 9, 2014

Area of Science:

Immunology
Genetics
Molecular Biology

Background:

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia and increased susceptibility to infections.
CVID patients exhibit diverse clinical, cellular, and immunological defects, with increased risks of autoimmune diseases and malignancies.
Recent discoveries reveal genetic defects in ICOS, TACI, BAFF-R, and CD19 contribute to the CVID phenotype, highlighting genetic heterogeneity.

Purpose of the Study:

To provide an overview of the molecular basis of Common variable immunodeficiency (CVID).
To discuss how genetic defects in key molecules disrupt B cell maturation and function.
To present data illustrating the link between CVID-associated molecular defects and the development of autoimmunity.

Main Methods:

Literature review of genetic defects associated with CVID.
Analysis of the roles of ICOS, TACI, BAFF-R, and CD19 in B cell development.
Presentation of experimental data linking molecular defects to autoimmune manifestations in CVID.

Main Results:

Identification of four genetic defects (ICOS, TACI, BAFF-R, CD19) underlying CVID.
Demonstration that deficiencies in these molecules impair B cell maturation and function at distinct stages.
Evidence suggesting a connection between specific molecular defects and the pathogenesis of autoimmunity in CVID patients.

Conclusions:

The genetic basis of CVID is highly variable, involving multiple genes crucial for B cell regulation.
Understanding the molecular mechanisms of these defects is essential for elucidating CVID pathogenesis.
Further research is needed to fully comprehend how these genetic variations lead to immune dysfunction and autoimmunity.