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- Title
Freeze-fracture analysis of cholesterol in muscle plasma membrane of Fukuyama-type congenital muscular dystrophy.
- Authors
Wakayama, Y.; Kumagai, T.; Jimi, T.; Shibuya, S.
- Abstract
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<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.
- Publication
Acta Neuropathologica, 1987, Vol 75, Issue 1, p46
- ISSN
0001-6322
- Publication type
Article
- DOI
10.1007/BF00686792