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Defective interferon-gamma production in ataxia-telangiectasia.

Authors: Paganelli, R  Capobianchi, MR  Matricardi, PM  Cioe, L  Seminara, R  Dianzani, F  Aiuti, F 
Citation: Paganelli R, etal., Clin Immunol Immunopathol. 1984 Sep;32(3):387-91.
Pubmed: (View Article at PubMed) PMID:6432389

The in vitro production of interferon-alpha and -gamma (IFN) by peripheral blood mononuclear cells from four patients with ataxia-telangiectasia was compared to that of healthy controls. Normal values of IFN-alpha were obtained in all cases. However, patients with ataxia-telangiectasia showed a great reduction or absence of IFN-gamma 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-gamma may contribute to the abnormalities of immune functions and immunoregulation observed in ataxia-telangiectasia, and it may represent an additional cause of the high incidence of viral infections and neoplasia in this disease.


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RGD Object Information
RGD ID: 8693328
Created: 2014-07-10
Species: All species
Last Modified: 2014-07-10
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.