Impairment of capping in lymphoblastoid cell lines of Duchenne patients indicates an intrinsic cellular defect.
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 confirmed. 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.
Baricordi
O R
OR
Istituto di Genetica Medica, Università di Ferrara, Italy.
Sensi
A
A
Balboni
A
A
Romeo
G
G
Rocchi
M
M
Melchiorri
L
L
Gandini
E
E
D000911:Antibodies, Monoclonal; D002461:Cell Line, Transformed; D006801:Humans; D007152:Immunologic Capping; D008214:Lymphocytes / Q000276:immunology*; D009136:Muscular Dystrophies / Q000235:genetics* / Q000276:immunology
D000911:Antibodies, Monoclonal