Freeze-fracture analysis of cholesterol in muscle plasma membrane of Fukuyama-type congenital muscular dystrophy.
We used digitonin in freeze-fracture analysis of the muscle plasma membrane cholesterol content in six patients with Fukuyama-type congenital muscular dystrophy and six control children. A significantly greater proportion of surface area was taken up by digitonin-cholesterol complexes in the patients (51.2% +/- 4.7%) than in controls (31.9% +/- 2.9%) (P less than 0.01). Since membrane cholesterol has a dynamic regulatory function in affecting the activity of membrane-bound proteins, the increased cholesterol content in the patients suggests a functional abnormality of the muscle plasma membrane in this disease.
Department of Medicine, Showa University Fujigaoka Hospital, Yokohama, Japan.
Y
Y
Wakayama
T
T
Kumagai
T
T
Jimi
S
S
Shibuya
D002462:Cell Membrane / Q000648:ultrastructure; D002648:Child; D002784:Cholesterol / Q000032:analysis*; D005614:Freeze Fracturing; D006801:Humans; D008563:Membrane Lipids / Q000032:analysis; D008854:Microscopy, Electron; D009132:Muscles / Q000473:pathology / Q000648:ultrastructure*; D009136:Muscular Dystrophies / Q000473:pathology*; D012016:Reference Values
D008563:Membrane Lipids; D002784:Cholesterol