Freeze fracture studies of muscle plasma membrane in human muscular dystrophy.
Freeze fracture analysis of intramembranous particle density in skeletal muscle plasma dystrophy 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 depletion of intramembranous particles including orthogonal arrays was noted in DMD while only orthogonal arrays were significantly decreased in FSH. No abnormalities were noted in MyD.
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002462:Cell Membrane / Q000648:ultrastructure*; D002648:Child; D002675:Child, Preschool; D005614:Freeze Fracturing; D006801:Humans; D008297:Male; D008855:Microscopy, Electron, Scanning; D009132:Muscles / Q000473:pathology / Q000648:ultrastructure*; D009136:Muscular Dystrophies / Q000473:pathology*; D009223:Myotonic Dystrophy / Q000473:pathology