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Defective Calcium‐Dependent Signal Transduction in T Lymphocytes of Ataxia‐Telangiectasia
Author(s) -
KONDO N.,
INOUE R.,
NISHIMURA S.,
KASAHARA K.,
KAMEYAMA T.,
MIWA Y.,
I P. R. LORENZO,
ORII T.
Publication year - 1993
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.1993.tb01692.x
Subject(s) - ataxia telangiectasia , phytohaemagglutinin , cd8 , cd3 , t cell , peripheral blood mononuclear cell , stimulation , signal transduction , endocrinology , immunology , microbiology and biotechnology , biology , medicine , chemistry , immune system , biochemistry , in vitro , dna , dna damage
T‐cell functions of two patients with ataxia‐telangiectasia were investigated. Patients with ataxia‐telangiectasia had reduced percentages of circulating CD3 + cells and CD4 + cells, although neither patient had a reduced percentage of circulating CD8 + cells. The proliferative responses and interleukin‐2 production of peripheral blood mononuclear cells to T‐cell mitogens were reduced in the patients. The intracellular calcium concentration in T cells or CD4 + cells from both patients was only slightly increased after phytohaemagglutinin stimulation. Moreover, the concentration after OKT3 stimulation was not or only slightly increased in T cells or CD4 + cells from both patients. Our results suggest that the functional defect of T cells is caused by defective Ca 2+ ‐dependent signal transduction through the CD3 complex of the surface in T cells of ataxia‐telangiectasia.