Immunoglobulin Production in Epstein-Barr Virus-Transformed Lymphoblastoid Cell Lines in Patients with Ataxia Telangiectasia

摘要

Peripheral blood lymphocytes from 7 patients with ataxia telangiectasia were transformed by Epstein-Barr virus, and lymphoblastoid cell lines (LCL) were established. Immunoglobulin levels in the supernatants of the LCL were measured in order to analyze immunological abnormalities, especially the low levels of serum IgA in these patients. We found that the geometric means of IgA production in the supernatants of the LCL from the 7 patients were significantly lower than those from 9 healthy controls (5.0 vs. 834 ng/ml, p < 0.001). These results indicate that the low levels of serum IgA in ataxia telangiectasia are attributable at least in part to an intrinsic abnormality of B cells.