Freeze-fracture electronmicroscopic analysis of plasma membranes of cultured muscle cells in duchenne dystrophy

Mitsuhiro Osame, Andrew G. Engel, Charles J. Rebouche, Robert E. Scott

Research output: Contribution to journalArticlepeer-review

25 Scopus citations

Abstract

Depletion of intramembranous particles has been reported in the muscle fiber plasma membrane in Duchenne dystrophy. We searched for a similar abnormality in plasma membranes of cultured muscle cells obtained from six patients with Duchenne dystrophy and six controls. A precise method for phase microscopic to freeze-fracture electronmicroscopic correlation was devised to identify the cell of origin of each replicated membrane face. For both control and patient cells, P-face particle density was higher in myotubes than in mononuclear cells. However, there were no significant differences between control and dystrophic cells when comparing P faces of myotubes, E faces of myotubes, P faces of single cells, or E faces of single cells. The frequency distribution of particle diameters was similar in P faces of control and Duchenne myotubes. Other structural features of the freeze-fractured plasma membrane also were similar.

Original languageEnglish (US)
Pages (from-to)972-979
Number of pages8
JournalNeurology
Volume31
Issue number8
DOIs
StatePublished - Aug 1981

ASJC Scopus subject areas

  • Clinical Neurology

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