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Freeze fracture studies of muscle plasma membrane in human muscular dystrophy
Authors:D. L. Schotland  E. Bonilla  Y. Wakayama
Affiliation:(1) Henry M. Watts, Jr., Neuromuscular Disease Research Center, University of Pennsylvania School of Medicine, 19104 Philadelphia, PA, USA;(2) Department of Neurology, University of Pennsylvania School of Medicine, 19104 Philadelphia, PA, USA;(3) Department of Neurology, Hospital of the University of Pennsylvania, 19104 Philadelphia, PA, USA
Abstract:
Summary Freeze fracture analysis of intramembranous particle density in skeletal muscle plasma membrane from 7 patients with Duchenne muscular dystrophy (DMD), 5 patients with facioscapulohumeral muscular dystrophy (FSH) and 5 patients with myotonic dystrophy (MyD) were carried out. Marked deplction of intramembranous particles including orthogonal arrays were significantly decreased in FSH. No abnormalities were noted in MyD.This work was supported by a Research Center Grant from the Muscular Dystrophy Association of America and by grants NS-08075, NS-14471 and 5 M01 RR00040 from the U.S. Public Health Service
Keywords:Freeze fracture  Membrane  Muscular dystrophy
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