Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Immunodeficiency Diseases01:25

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...
Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

Overview
Hedgehog Signaling Pathway02:33

Hedgehog Signaling Pathway

The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...
T Cell Activation and Clonal Selection01:22

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.
Naive T cells that have not yet encountered an antigen express two primary CD...
Role Of Notch Signalling In Intestinal Stem Cell Renewal01:12

Role Of Notch Signalling In Intestinal Stem Cell Renewal

Notch signaling was first discovered in Drosophila melanogaster, where it is involved in cell lineage differentiation. Notch signaling regulates the maintenance and differentiation of intestinal stem cells or ISCs by controlling the expression of atonal homolog 1 or Atoh1. Atoh1 directs cells to differentiate into secretory cells.
Direct cell-to-cell contact is needed for the activation of Notch signaling. The signal is initiated when a notch ligand binds to a receptor on an adjacent cell, also...
Humoral Immune Responses01:36

Humoral Immune Responses

Overview

You might also read

Related Articles

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

Sort by
Same author

Early identification of delayed-onset ADA deficiency: The case for expanded first-tier newborn screening.

Journal of human immunity·2026
Same author

Clinical Evaluation of Pharmacokinetics, Efficacy, and Safety of a 10% Immunoglobulin in Primary Immunodeficiency Disease (CARES10 Study).

Advances in therapy·2026
Same author

Atopy and immune dysregulation among patients with chronic granulomatous disease.

Frontiers in immunology·2026
Same author

Gene alterations in inborn errors of immunity and their presence in cancers: Implications for oncogenesis, progression, and outcomes.

Human immunology·2026
Same author

Hematopoietic transplantation for PNP deficiency: it's time.

Blood·2026
Same author

Improved outcome of HSCT in STAT1 gain-of-function disease following JAK inhibition bridging.

Journal of human immunity·2025

Related Experiment Video

Updated: Jul 19, 2026

Stem-cell Based Engineered Immunity Against HIV Infection in the Humanized Mouse Model
11:38

Stem-cell Based Engineered Immunity Against HIV Infection in the Humanized Mouse Model

Published on: July 2, 2016

Human T cell immunodeficiency: when signal transduction goes wrong.

Eyal Grunebaum1, Nigel Sharfe, Chaim M Roifman

  • 1Division of Immunology/Allergy and the Infection, Immunity, Injury and Repair Program, The Research Institute and The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Immunologic Research
|September 28, 2006
PubMed
Summary

Severe combined immunodeficiency (SCID) is a fatal infant disease. Research identified molecular defects in T cell development, advancing understanding of immune reconstitution and function.

More Related Videos

Generation of Human Alloantigen-specific T Cells from Peripheral Blood
09:47

Generation of Human Alloantigen-specific T Cells from Peripheral Blood

Published on: November 21, 2014

Humanized NOD/SCID/IL2rγnull (hu-NSG) Mouse Model for HIV Replication and Latency Studies
07:10

Humanized NOD/SCID/IL2rγnull (hu-NSG) Mouse Model for HIV Replication and Latency Studies

Published on: January 7, 2019

Related Experiment Videos

Last Updated: Jul 19, 2026

Stem-cell Based Engineered Immunity Against HIV Infection in the Humanized Mouse Model
11:38

Stem-cell Based Engineered Immunity Against HIV Infection in the Humanized Mouse Model

Published on: July 2, 2016

Generation of Human Alloantigen-specific T Cells from Peripheral Blood
09:47

Generation of Human Alloantigen-specific T Cells from Peripheral Blood

Published on: November 21, 2014

Humanized NOD/SCID/IL2rγnull (hu-NSG) Mouse Model for HIV Replication and Latency Studies
07:10

Humanized NOD/SCID/IL2rγnull (hu-NSG) Mouse Model for HIV Replication and Latency Studies

Published on: January 7, 2019

Area of Science:

  • Immunology
  • Molecular Biology
  • Genetics

Background:

  • Severe combined immunodeficiency (SCID) is a group of fatal infant diseases requiring stem cell replacement.
  • Effective management involves rapid diagnosis and treatment for immune reconstitution.

Purpose of the Study:

  • To identify molecular defects causing SCID.
  • To advance the understanding of T cell development, function, and homeostasis.

Main Methods:

  • Extensive immunological, biochemical, and genetic studies of patient samples.
  • Comparative analysis of human and murine T cell development.

Main Results:

  • Identified molecular defects in SCID, including ZAP-70, IL2R alpha, and CD3delta.
  • Advanced understanding of signal-transducing proteins in T cell maturation.

Conclusions:

  • Discoveries have significantly improved the understanding of T cell development and function in humans.
  • Research contributes to better SCID management and immune reconstitution strategies.