Summary
Rats with electromyographic symptoms of “myotonia” and biochemical alterations (i.e. reduced hexokinase activity and alterations in the fatty acid components of phospholipids in skeletal muscles) after prolonged application of 20.25-diazacholesterol failed to show any generalized structural abnormalities in muscle fibers of the thigh. No light or electron microscopic changes responsible for the functional disturbance of myotonia were identified in our experimental animals. Occasionally hyaline degeneration, phagocytosis and vacuolar degeneration of single muscle fibers could be observed which presumably were caused by a non-specific, toxic effect of 20.25-diazacholesterol, or triparanol respectively. New ultrastructural abnormalities, not seen before, could be demonstrated in vacuolated muscle fibers.
Zusammenfassung
Nach Applikation von 20,25-Diazacholesterin traten bei Ratten im Verlauf von mehreren Wochen elektromyographische Symptome der “Myotonie” und biochemisch nachweisbare Störungen des Stoffwechsels auf. Letztere bestanden in einer Reduktion der Aktivität der Hexokinase und in Veränderungen des Fettsäurengehaltes der Phosphatide der Skeletmuskulatur. Licht- und elektronenmikroskopisch waren keine Strukturveränderungen feststellbar, die alle Muskelfasern gemeinsam betrafen. Ein morphologisches Substrat der Myotonie konnte also entsprechend den negativen Befunden bei menschlichem Biopsiematerial von Myotonie-Patienten nicht mit Sicherheit nachgewiesen werden. Wohl aber fanden sich, wenn auch spärlich, vermutlich unspezifisch-toxische Faserveränderungen wie vacuoläre und hyaline Degenerationen von Muskelfasern. Neue elektronenmikroskopische Befunde konnten vor allem bei der vacuoligen Fasererkrankung erhoben werden.
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Schröder, J.M., Kuhn, E. Zur Ultrastruktur der Muskelfaser bei der experimentellen „Myotonie“ mit 20,25-Diazacholesterin. Virchows Archiv Abteilung A Pathologische Anatomie 344, 181–195 (1968). https://doi.org/10.1007/BF00547886
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DOI: https://doi.org/10.1007/BF00547886