Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

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...

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...

Introduction to Innate and Adaptive Immunity

Introduction to Innate and Adaptive Immunity

The human immune system is a complex defense mechanism that protects the body from harmful pathogens and foreign substances. It comprises two crucial components: innate and adaptive immunity.
Innate immunity is the body's natural, nonspecific defense system that acts quickly to protect against pathogens. It incorporates physical barriers like skin and mucous membranes and cellular elements such as phagocytes and natural killer cells. This part of our immune system provides an immediate,...

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,...

Humoral Immune Responses

Humoral Immune Responses

Primary Lymphoid Organs

Primary Lymphoid Organs

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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Correction to: Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity.

Journal of clinical immunology·2019

Same author

Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity.

Journal of clinical immunology·2019

Same author

Human inborn errors of immunity to infection affecting cells other than leukocytes: from the immune system to the whole organism.

Current opinion in immunology·2019

Same author

Recurrent Salmonella typhi Infection and Autoimmunity in a Young Boy with Complete IL-12 Receptor β1 Deficiency.

Journal of clinical immunology·2019

Same author

Homozygosity for <i>TYK2</i> P1104A underlies tuberculosis in about 1% of patients in a cohort of European ancestry.

Proceedings of the National Academy of Sciences of the United States of America·2019

Same author

SeqTailor: a user-friendly webserver for the extraction of DNA or protein sequences from next-generation sequencing data.

Nucleic acids research·2019

Search research articles

Related Experiment Video

Updated: Jul 13, 2026

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Published on: December 6, 2014

Primary immunodeficiencies: a field in its infancy.

Jean-Laurent Casanova¹, Laurent Abel

¹Laboratory of Human Genetics of Infectious Diseases, Institut National de la Santé et de la Recherche Médicale, U550, Paris, France. casanova@necker.fr

Science (New York, N.Y.)

|August 4, 2007

Summary

Primary immunodeficiencies, or inborn errors of immunity, are redefining their scope beyond rare, monogenic conditions. Research reveals these immune system disorders are more common and diverse than previously understood, impacting human medicine.

More Related Videos

Mapping Infant Immunity with Minimal Input: Integrative Single-Cell and Multiomic Profiling

Mapping Infant Immunity with Minimal Input: Integrative Single-Cell and Multiomic Profiling

Published on: April 3, 2026

Isolation and Transplantation of Different Aged Murine Thymic Grafts.

Isolation and Transplantation of Different Aged Murine Thymic Grafts.

Published on: May 13, 2015

Related Experiment Videos

Last Updated: Jul 13, 2026

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Published on: December 6, 2014

Mapping Infant Immunity with Minimal Input: Integrative Single-Cell and Multiomic Profiling

Mapping Infant Immunity with Minimal Input: Integrative Single-Cell and Multiomic Profiling

Published on: April 3, 2026

Isolation and Transplantation of Different Aged Murine Thymic Grafts.

Isolation and Transplantation of Different Aged Murine Thymic Grafts.

Published on: May 13, 2015

Area of Science:

Immunology
Genetics
Human Medicine

Background:

Primary immunodeficiencies (PIs) were historically defined as rare, familial, monogenic, recessive disorders affecting leukocyte subsets and causing severe infections in infancy.
This traditional view is being challenged by accumulating exceptions and a broader understanding of immune system variations.

Purpose of the Study:

To reflect the paradigm shift in the definition and scope of primary immunodeficiencies.
To highlight the expanding understanding of inborn errors of immunity.

Main Methods:

Review of existing literature and case studies.
Analysis of exceptions to the conventional definition of PIs.

Main Results:

The definition of primary immunodeficiencies is undergoing revision, expanding beyond the initial narrow scope.
A significant number of individuals are now understood to have PIs, challenging the notion of rarity.

Conclusions:

The field of inborn errors of immunity is rapidly evolving.
Understanding the diversity of PIs is crucial for advancing human medicine and studying immunity in its natural state.