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 Experiment Videos

Canine X-linked severe combined immunodeficiency.

P J Felsburg1, B J Hartnett, P S Henthorn

  • 1Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, USA. felsburg@vet.upenn.edu

Veterinary Immunology and Immunopathology
|October 3, 1999
PubMed
Summary

Canine X-linked severe combined immunodeficiency (XSCID) results from mutations in the common gamma (gamma c) chain. This leads to severe immune dysfunction, characterized by stunted growth and recurrent infections in affected dogs.

Related Concept Videos

You might also read

Related Articles

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

Sort by
Same author

Clinical characteristics and outcome in dogs with small cell T-cell intestinal lymphoma.

Veterinary and comparative oncology·2018
Same author

Feline Respiratory Extramedullary Plasmacytoma with Lymph Node Metastasis and Intrahistiocytic Amyloid.

Journal of comparative pathology·2016
Same author

Canine Nervous System Lymphoma Subtypes Display Characteristic Neuroanatomical Patterns.

Veterinary pathology·2016
Same author

Retrospective characterisation of solitary cutaneous histiocytoma with lymph node metastasis in eight dogs.

The Journal of small animal practice·2016
Same author

Cutaneous Lymphoma at Injection Sites: Pathological, Immunophenotypical, and Molecular Characterization in 17 Cats.

Veterinary pathology·2016
Same author

Clonality Testing in Veterinary Medicine: A Review With Diagnostic Guidelines.

Veterinary pathology·2016

Area of Science:

  • Immunology
  • Genetics
  • Veterinary Medicine

Background:

  • Canine X-linked severe combined immunodeficiency (XSCID) is a critical genetic disorder affecting immune system development.
  • Mutations in the common gamma (gamma c) chain subunit disrupt cytokine receptor function, leading to severe immunodeficiency.
  • Affected dogs exhibit stunted growth and susceptibility to recurrent infections, rarely surviving past four months.

Purpose of the Study:

  • To investigate the cellular and molecular basis of canine XSCID.
  • To elucidate the role of the common gamma (gamma c) chain in canine lymphocyte development and function.
  • To characterize the immunophenotype and functional defects in T and B cells of XSCID dogs.

Main Methods:

  • Analysis of thymus pathology and lymphocyte populations (T cells, B cells) in affected dogs.

Related Experiment Videos

  • Flow cytometry to assess T cell phenotypes (CD45RA, CD4, CD8) and thymocyte subsets.
  • In vitro proliferation assays to evaluate T cell receptor and B cell mitogen responses.

    • Main Results:

    • XSCID dogs display a severely hypoplastic thymus with altered thymocyte subset proportions.
    • Peripheral T cell numbers are significantly reduced, with a rapid switch to a CD45RA- phenotype.
    • T cells exhibit a profound defect in proliferation due to non-functional IL-2 receptors.
    • B cells fail to class-switch from IgM to IgG production in response to T cell-dependent antigens.

    Conclusions:

    • A functional gamma c chain is essential for normal T and B cell function and development in dogs.
    • Early T cell development is critically dependent on the gamma c chain.
    • Canine B cell development can proceed independently of the gamma c chain, but antibody class-switching is impaired.