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

Defective CD2 T cell pathway activation in common variable immunodeficiency (CVID)

S Zielen1, T J Dengler, P Bauscher

  • 1Department of Paediatrics, J.W. Goethe-Universität Frankfurt, Germany.

Clinical and Experimental Immunology
|May 1, 1994
PubMed
Summary
This summary is machine-generated.

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

Characteristics of children with severe preschool asthma prior to starting the TIPP study.

Frontiers in pediatrics·2025
Same author

Response to Correspondence to "Short-course subcutaneous treatment with PQ Grass strongly improves symptom and medication scores in grass allergy".

Allergy·2024
Same author

Short-course subcutaneous treatment with PQ Grass strongly improves symptom and medication scores in grass allergy.

Allergy·2023
Same author

Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis.

Canadian journal of gastroenterology & hepatology·2023
Same author

Correction to: Neurofilament Light Chain is a Biomarker of Neurodegeneration in Ataxia Telangiectasia.

Cerebellum (London, England)·2021
Same author

Neurofilament Light Chain Is a Biomarker of Neurodegeneration in Ataxia Telangiectasia.

Cerebellum (London, England)·2021

Common variable immunodeficiency (CVID) patients show impaired T cell responses due to defects in the CD2 pathway. This T cell deficiency may stem from an early signaling defect before protein kinase C activation.

Area of Science:

  • Immunology
  • Cellular Biology
  • Clinical Medicine

Background:

  • Clonal T cell expansion requires T cell receptor (TCR) and secondary signals like CD2, CD4, and CD28.
  • CD2/CD58 interaction is crucial for primary immune responses and antibody production.
  • Defective CD2 pathway activation has not been previously demonstrated in Common Variable Immunodeficiency (CVID).

Purpose of the Study:

  • To investigate the CD2 pathway activation in T cells from patients with CVID.
  • To identify potential defects in T cell signaling pathways contributing to CVID.

Main Methods:

  • Assessed T cell proliferation in CVID patients and controls using various stimuli.
  • Monitored costimulatory effects of monocytes on CD2-triggered proliferation.
  • Evaluated the role of IL-1 as a comitogenic factor in CD2 pathway activation.

Related Experiment Videos

  • Utilized monoclonal antibodies (MoAbs) for CD2, CD45, and TCR-CD3 stimulation.
  • Tested the effect of phorbol ester on T cell responses.

    • Main Results:

    • CVID patients exhibited significantly impaired CD2-triggered T cell proliferation compared to controls.
    • The costimulatory effect of monocytes on CD2-triggered proliferation was reduced in CVID patients.
    • IL-1 showed a defective amplifier function for the CD2 pathway in most CVID patients.
    • T cell proliferation was severely depressed in CVID patients when stimulated with CD2 plus CD45 MoAb.
    • Phytohaemagglutinin (PHA) responsiveness was markedly reduced in CVID patients, while TCR-CD3 response remained unaffected.

    Conclusions:

    • The T cell deficiency in CVID is partly attributed to defects in the CD2 pathway.
    • An early signal-transducing defect, proximal to protein kinase C (PKC) activation, is suggested in CVID patients.
    • Restoration of T cell responses by direct PKC activation indicates a defect upstream of this enzyme.