Impairment of capping in lymphoblastoid cell lines of Duchenne patients indicates an intrinsic cellular defect

Hum Genet. 1989 Oct;83(3):217-9. doi: 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 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.

MeSH terms

  • Antibodies, Monoclonal
  • Cell Line, Transformed
  • Humans
  • Immunologic Capping*
  • Lymphocytes / immunology*
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / immunology

Substances

  • Antibodies, Monoclonal