Properties of acetylcholine-receptor activation in human Duchenne muscular dystrophy myotubes

E. Mancinelli, A. Sardini, A. D'Aumiller, G. Meola, G. Martucci, G. Cossu, E. Wanke

Research output: Contribution to journalArticlepeer-review

Abstract

In human myotubes cultured from biopsies of normal subjects and dystrophic patients we investigated, with the patch-clamp technique, the activation properties of the nicotinic acetylcholine receptor (AChoR) in the presence of acetylcholine and suberyldicholine. The single-channel conductance and the lifetime of the openings were not found to differ. In contrast, the average frequency of openings was about four times higher in Duchenne muscular dystrophy (DMD) myotubes in the presence of equal amounts of acetylcholine, but not of suberyldicholine. The most reasonable conclusion from this observation is that the behaviour of the AChoR is not altered in DMD cells but that there is a greater average concentration of ACho molecules present around AChoRs. This leads to the tentative conclusion that the activity of the enzyme acetylcholinesterase (AChoE) is impaired by some unknown mechanism in the dystrophic myotube.

Original languageEnglish
Pages (from-to)247-257
Number of pages11
JournalProceedings of the Royal Society B: Biological Sciences
Volume237
Issue number1287
Publication statusPublished - 1989

ASJC Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Agricultural and Biological Sciences (miscellaneous)

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