Impairment of capping in lymphoblastoid cell lines of Duchenne patients indicates an intrinsic cellular defect
- 1 October 1989
- journal article
- research article
- Published by Springer Nature in Human Genetics
- Vol. 83 (3) , 217-219
- https://doi.org/10.1007/bf00285158
Abstract
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.Keywords
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