TY - JOUR
T1 - Defective interferon-γ production in ataxia-telangiectasia
AU - Paganelli, R.
AU - Capobianchi, M. R.
AU - Matricardi, P. M.
AU - Cioè, L.
AU - Seminara, R.
AU - Dianzani, F.
AU - Aiuti, F.
PY - 1984
Y1 - 1984
N2 - The in vitro production of interferon-α and -γ (IFN) by peripheral blood mononuclear cells from four patients with ataxia-telangiectasia was compared to that of healthy controls. Normal values of IFN-α were obtained in all cases. However, patients with ataxiatelangiectasia showed a great reduction or absence of IFN-γ production after induction with either staphylococcal enterotoxin B or galactose oxidase. This defect was accompanied by the absence of secretion of another lymphokine, namely, interleukin 2 (IL-2), in one case. Lymphoproliferative response to phytohemagglutinin (PHA) was severely depressed in all patients. Near normal values of T lymphocytes were found, but the ratio of OKT4+ OKT8+ subsets was reduced in most patients, due to a decrease of OKT4+ lymphocytes. Deficiency of IFN-γ may contribute to the abnormalities of immune functions and immunoregulation observed in ataxia-teleangiectasia, and it may represent an additional cause of the high incidence of viral infections and neoplasia in this disease.
AB - The in vitro production of interferon-α and -γ (IFN) by peripheral blood mononuclear cells from four patients with ataxia-telangiectasia was compared to that of healthy controls. Normal values of IFN-α were obtained in all cases. However, patients with ataxiatelangiectasia showed a great reduction or absence of IFN-γ production after induction with either staphylococcal enterotoxin B or galactose oxidase. This defect was accompanied by the absence of secretion of another lymphokine, namely, interleukin 2 (IL-2), in one case. Lymphoproliferative response to phytohemagglutinin (PHA) was severely depressed in all patients. Near normal values of T lymphocytes were found, but the ratio of OKT4+ OKT8+ subsets was reduced in most patients, due to a decrease of OKT4+ lymphocytes. Deficiency of IFN-γ may contribute to the abnormalities of immune functions and immunoregulation observed in ataxia-teleangiectasia, and it may represent an additional cause of the high incidence of viral infections and neoplasia in this disease.
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U2 - 10.1016/0090-1229(84)90282-4
DO - 10.1016/0090-1229(84)90282-4
M3 - Article
C2 - 6432389
AN - SCOPUS:0021127724
VL - 32
SP - 387
EP - 391
JO - Clinical Immunology and Immunopathology
JF - Clinical Immunology and Immunopathology
SN - 0090-1229
IS - 3
ER -