Virchows Archiv A

, Volume 348, Issue 1, pp 89–104 | Cite as

Zur Ultrastruktur der „Target-Fasern“

  • Masanori Tomonaga
  • Elfriede Sluga
Article

Zusammenfassung

Die Ultrastruktur der Target-Fasern (W. K. Engel, 1961) in Muskelfasern bei neurogener Atrophie wurden untersucht. Die dreizonige Struktur der Targetformation zeigt in der zentralen Zone elektronendichte Zopfbildungen, für die eine Kontinuität mit fragmentierten Z-Streifen in der Zwischenzone nachgewiesen werden konnte. Es besteht keine Periodizität wie in den Stäbchen der Nemaline-Myopathie. In der Zwischenzone finden sich Z-Streifen-Störungen, gefolgt von Ordnungsstörungen der Sarkomeren. Vermehrte sarkoplasmatische Strukturen sind vorhanden. Die Außenzone zeigt wenig pathologische Veränderungen. Diese ultrastrukturellen Befunde weisen eher auf eine Veränderung von myopathischem Typ hin. Pathogenetisch wird ein intramuskulärer Degenerationsprozeß bei inkompletter Denervation vermutet. Targetoid-Fasern wurden untersucht und als Früh- oder Spätstadien der Target-Fasern gedeutet. Die Beziehung der Target-Fasern zu den Faserveränderungen des Central Core Disease werden diskutiert.

The ultrastructure of „target fibers“

Summary

The ultrastructure of the target fibers (W. K. Engel, 1961) in muscle fibers of neurogenic atrophy was investigated. The target formation with three concentric zones showed in its central zone disorganized myofilaments with electron dense amorphous material that revealed a continuity with fragmented Z-disks in the intermediate zone. A periodicity, as in the rods of nemaline myopathy, was not observed. In the intermediate zone the Z-disks were disintegrated and associated with disorganized sarcomeres. Sarcoplasmic components were usually increased. The outer zone showed only minimal pathologic changes. These ultrastructural changes are of the myopathic type and suggest pathogenetically some intramuscular degenerative process presumably after a long-standing incomplete denervation. The targetoid fibers were also investigated. Some of them occurred in early or late stages of target formation. The ultrastructural similarity of the target fibers with the fiber changes in central core disease is suspected.

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Copyright information

© Springer-Verlag 1969

Authors and Affiliations

  • Masanori Tomonaga
    • 1
  • Elfriede Sluga
    • 1
  1. 1.Neurologisches Institut der Universität WienÖsterreich

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