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Updated: May 28, 2026

Characterization of Thymus-dependent and Thymus-independent Immunoglobulin Isotype Responses in Mice Using Enzyme-linked Immunosorbent Assay

Characterization of Thymus-dependent and Thymus-independent Immunoglobulin Isotype Responses in Mice Using Enzyme-linked Immunosorbent Assay

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[Advancements in the Inborn Errors of Immunity].

Ori Toker¹, Raz Somech²

¹Allergy and Clinical Immunology Unit, Shaare Zedek Medical Centre, Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel, The Israeli Association for Allergy and Clinical Immunology.

|May 27, 2026

Summary

Inborn errors of immunity (IEI) are genetic disorders affecting the immune system, previously known as primary immunodeficiencies. These conditions, encompassing 559 distinct clinical entities, are more common than previously believed.

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Area of Science:

Immunology
Genetics
Clinical Medicine

Background:

The immune system protects against diseases but damage can lead to immune disorders.
Genetic defects underlie primary immune system disorders, termed inborn errors of immunity (IEI).
IEI encompasses 559 conditions, previously called primary immunodeficiencies (PID).

Purpose of the Study:

To introduce the concept and scope of inborn errors of immunity (IEI).
To highlight the genetic basis and diverse manifestations of IEI.
To underscore the prevalence and clinical significance of IEI.

Main Methods:

Review of immunological and genetic principles.
Analysis of the classification and nomenclature of immune system disorders.
Epidemiological data on the prevalence of congenital immune diseases.

Main Results:

Inborn errors of immunity (IEI) represent a broad spectrum of genetic conditions.
These disorders result from defects in immune system components, leading to varied clinical presentations.
The prevalence of IEI is estimated at 1:1,200, indicating they are not rare.

Conclusions:

The term IEI replaces PID, reflecting a deeper understanding of genetic immune disorders.
IEI are caused by genetic defects and can manifest as immune deficiency or overactivity.
IEI are a significant group of disorders with a notable prevalence in the general population.