[In vitro study of fibroblasts from patients with Duchenne muscular dystrophy. Evaluation of capping and ultrastructural aspect].
There have been conflicting studies of lymphocyte capping from patients with Duchenne Muscular Dystrophy. We have evaluated the proportion of capped fibroblasts of 10 patients with Duchenne Muscular Dystrophy. The results, compared with 15 normal controls, showed that the reduction of fibroblast capping is correlated to age patients. Additional studies, decreased number of retracted fibroblasts after colchicine incubation and ultrastructural observations, suggest that capping deficit would be the consequence of microtubular system alteration. This late phenomenon may be secondary to the primary membrane defect.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000375:Aging; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000494:pharmacology; D003208:Concanavalin A / Q000494:pharmacology; D005347:Fibroblasts / Q000187:drug effects / Q000473:pathology*; D006801:Humans; D007223:Infant; D008247:Lysosomes / Q000473:pathology; D008854:Microscopy, Electron; D008870:Microtubules / Q000473:pathology; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000473:pathology*
D003208:Concanavalin A; D003078:Colchicine
Etude in vitro de fibroblastes de malades atteints de myopathie de Duchenne: évaluation du capping et aspect ultrastructural.