DNA Synthesis in vitro in Leukocytes from Patients with Ataxia telangiectasia

Transformation of Blood Lymphocytes in Ataxia telangiectasia

“…In contrast, N aspitz et al [45] found no evidence for a deficiency of such a humoral factor. The degree of impairment of the lymphocyte transformation has been related to the degree of immunological deficiency, particularly of the delayed hypersenitivity reactions [3,12,21,28,34,38,45,46,47].…”

“…In contrast, N aspitz et al [45] found no evidence for a deficiency of such a humoral factor. The degree of impairment of the lymphocyte transformation has been related to the degree of immunological deficiency, particularly of the delayed hypersenitivity reactions [3,12,21,28,34,38,45,46,47]. It has been proposed that the lower than normal transformation is due to the presence of a large number of immunologically incompetent cells which do not transform, and of only relatively few immunologically competent cells which are capable of undergoing transformation [45].…”

“…Several investigators have demonstrated in recent years that blood lymphocytes from patients with ataxia telangiectasia show varying degrees of impairment in their in vitro transformation by phytohemagglutinin (PPHA), as compared to that of normal human blood lymphocytes [3,21,22,26,27,28,30,34,38,45,47,56], Most investi gators attribute this impairment to a defect of the lymphocytes [3,27,28,34,45], while others have presented evidence suggesting that, in addition to such a cellular defect, it may be in part due to a deficiency of a plasma factor [38,47]. In contrast, N aspitz et al [45] found no evidence for a deficiency of such a humoral factor.…”

“…The degree of impairment of the lymphocyte transformation has been related to the degree of immunological deficiency, particularly of the delayed hypersenitivity reactions [3,12,21,28,34,38,45,46,47]. It has been proposed that the lower than normal transformation is due to the presence of a large number of immunologically incompetent cells which do not transform, and of only relatively few immunologically competent cells which are capable of undergoing transformation [45]. It is possible, however, that the transforming lymphocytes in ataxia telangiectasia may not respond to PPHA as do normal lymphocytes, since we and others have found them to be abnormal, exhibiting numerous chromosome breaks [27,28].…”

Immunocompetent Cells in Man

“…to allow the cells to digest. The dissolved contents of the tubes were then transferred to scintillation counting vials, 15 ml of scintillation solution were added, and the 3H content of the vials was determined in a Packard Model 4000 Liquid Scintillation Counter [7],…”