Impairment of capping in lymphoblastoid cell lines of Duchenne patients indicates an intrinsic cellular defect |
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Authors: | O R Baricordi A Sensi A Balboni G Romeo M Rocchi L Melchiorri E Gandini |
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Institution: | (1) Istituto di Genetica Medica, Università di Ferrara, Via Luigi Borsari, 46, I-44100 Ferrara, Italy;(2) Laboratorio di Genetica Molecolare, Istituto G. Gaslini, Via 5 Maggio, 39, I-16148 Genova, Italy |
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Abstract: | Summary Duchenne muscular dystrophy (DMD) is a lethal sex-linked degenerative disorder of the muscle in man. Generalized cell membrane abnormalities seem to be involved in the pathogenesis of the disease; in particular, the impairment of lymphocyte capping capacities has been repeatedly confimed. To clarify whether capping impairment is a consequence of factors related to the activity of the disease or an expression of an intrinsic cellular defect, we have investigated the capping capacities of DMD EBV-transformed cell lines. The results indicate a significant impairment of capping capacity in cultured cell lines, providing evidence for an intrinsic cell deficiency in DMD. |
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