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@article{192404,
author = {Thon, Vojtěch and Wolf, HM. and Sasgary, M. and Litzman, J. and Samstag, A. and Hauber, I. and Lokaj, J. and Eibl, MM.},
article_number = {3},
keywords = {common variable immunodeficiency; activation; costimulation; T cell receptor; CD40 ligand},
language = {eng},
issn = {0009-9104},
journal = {Clin.Exp.Immunol.},
title = {Defective integration of activating signals derived from the T cell receptor (TCR) and costimulatory molecules in both CD4+ and CD8+ T Lymphocytes of Common Variable immunodeficiency (CVID) Patients},
volume = {31},
year = {1997}
}
TY - JOUR
ID - 192404
AU - Thon, Vojtěch - Wolf, HM. - Sasgary, M. - Litzman, J. - Samstag, A. - Hauber, I. - Lokaj, J. - Eibl, MM.
PY - 1997
TI - Defective integration of activating signals derived from the T cell receptor (TCR) and costimulatory molecules in both CD4+ and CD8+ T Lymphocytes of Common Variable immunodeficiency (CVID) Patients
JF - Clin.Exp.Immunol.
VL - 31
IS - 3
SP - 174
EP - 174
SN - 00099104
KW - common variable immunodeficiency
KW - activation
KW - costimulation
KW - T cell receptor
KW - CD40 ligand
N2 - CVID is characterized by hypogammaglobulinaemia and impaired antibody production. Previous studies demonstrated defects at the T cell level. In the present study the response of purified CD4(+) and CD8(+) T lymphocytes to stimulation with anti-TCR monoclonal antibody (the first signal) in combination with anti-CD4 or anti-CD8, anti-CD2 and anti-CD28 MoAbs (the costimulatory signals) was investigated. Both CD4(+) and CD8(+)T cells from the patients showed significantly reduced IL-2 release following stimulation via TCR and costimulation via CD4 or CD8 and CD2, respectively. However, normal IL-2 production following TCR plus phorbol myristate acetate (PMA) costimulation and normal expression of an early activation marker, CD69, after TCR + CD28 stimulation indicated that TCR was able to transduce a signal. Furthermore, both IL-2 and IL-4 release were impaired in CD4(+) lymphocytes following TCR+CD28 stimulation. In addition,stimulation via TCR+CD28 resulted in significantly decreased expression of CD40 Ligand in the patients. These results suggest that the integration of activating signals derived from the TCR and costimulatory molecules is defective in CVID patients; the defect is not confined to costimulation via a single molecule, or restricted to cells producing Th1-type cytokines such as IL-2, and is expressed in both CD4(+) and CD8(+) T cell subsets.
ER -
THON, Vojtěch, HM. WOLF, M. SASGARY, J. LITZMAN, A. SAMSTAG, I. HAUBER, J. LOKAJ and MM. EIBL. Defective integration of activating signals derived from the T cell receptor (TCR) and costimulatory molecules in both CD4+ and CD8+ T Lymphocytes of Common Variable immunodeficiency (CVID) Patients. \textit{Clin.Exp.Immunol.}. 1997, vol.~31, No~3, p.~174-181. ISSN~0009-9104.
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