Skip to main content
Log in

Desmin and actin associated with cytoplasmic bodies in skeletal muscle fibers: immunocytochemical and fine structural studies, with a note on unusual 18- to 20-nm filaments

  • Regular Papers
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

In a fine structural and immunocytochemical study, the latter performed on semithin sections of epoxy resin embedded skeletal muscle fibers, three types of cytoplasmic bodies were identified in a case of cytoplasmic body myopathy: (1) The first type, the classical type, showed a central core and a light halo with radiating actin filaments at the periphery. (2) The second type, the spheroid body was characterized by irregularly arranged granular masses associated with intermediate filaments. Desmin immunoreactivity occurred in the central and peripheral parts, where filaments of intermediate size were visualized by electron microscopy. Desmin immunoreactivity was noted also at the Z-bands of striated annulets, within areas of disordered myofibrils, such as sarcoplasmic masses, and in atrophic muscle fibers. (3) The third type of the cytoplasmic body was composed mainly of large masses of uneven granularity and electron density. The center of this type reacted to anti-actin antibody suggesting that the 5- to 6-nm filaments, which ultrastructurally proved to be a major component, were of the actin type. By contrast, neither intermediate filaments nor actin microfilaments were found by electron microscopy in cytoplasmic bodies in a second case where no immunoreaction to desmin or actin occurred. Anti-vimentin antibody stained only the cytoplasm of endomysial cells, but not the inclusion bodies. Some other, unusual inclusions with 18- to 20-nm tubulo-filamentous structures have to be distinguished from the various types of filaments in cytoplasmic bodies. It is concluded, that pleomorphism and heterogeneity of “cytoplasmic bodies” have to be taken into consideration when classifying cytoplasmic body myopathies.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Carpenter S, Karpati G (1984) Pathology of skeletal muscle. Churchill Livingstone, New York Edinburgh London Melbourne, p 258, p 265

    Google Scholar 

  2. Chou SM, Mizuno Y (1986) Induction of spheroid cytoplasmic bodies in a rat muscle by local tetanus. Muscle Nerve 9:455–464

    Google Scholar 

  3. Clark JR, D'Agostino AN, Wilson J, Brooks RR, Cole GC (1978) Autosomal dominant myofibrillar inclusion body myopathy: clinical, histologic, histochemical, and ultrastructural characteristics (abstract). Neurology 28:399

    Google Scholar 

  4. Edström L, Thornell LE, Eriksson A (1980) A new type of hereditary distal myopathy with characteristic sarcoplasmic bodies and intermediate (skeletin) filaments. J Neurol Sci 47:171–190

    Google Scholar 

  5. Engel WK (1962) The essentiality of histo- and cytochemical studies of skeletal muscle in the investigation of neuromuscular disease. Neurology 12:778–794

    Google Scholar 

  6. Fidzianska A, Goebel HH, Osborn M, Lenard HG, Osse G, Langenbeck U (1983) Mallory body-like inclusions in a hereditary congenital neuromuscular disease. Muscle Nerve 6:195–200

    Google Scholar 

  7. Goebel HH, Müller J, Gillen HW, Merrit AD (1978) Autosomal dominant “spheroid body myopathy”. Muscle Nerve 1:14–26

    Google Scholar 

  8. Goebel HH, Schloon H, Lenard HG (1981) Congenital myopathy with cytoplasmic bodies. Neuropediatrics 12:166–180

    Google Scholar 

  9. Granger BL, Lazarides E (1979) Desmin and vimentin coexist at the periphery of the myofibril Z-disc. Cell 18:1053–1063

    Google Scholar 

  10. Halbig L, Gutmann L, Goebel HH, Brick JF, Schochet S (1988) Ultrastructural pathology in emetine-induced myopathy. Acta Neuropathol (Berl) 75:577–582

    Google Scholar 

  11. Jerusalem F, Ludin H, Bischoff A, Hartmann G (1979) Cytoplasmic body neuromyopathy presenting a respiratory failure and weight loss. J Neurol Sci 41:1–9

    Google Scholar 

  12. Kinoshita M, Satoyoshi E, Suzuki Y (1975) Atypical myopathy with myofibrillar aggregates. Arch Neurol 32:417–420

    Google Scholar 

  13. Lazarides E, Hubbard BD (1976) Immunological characterization of the subunit of the 100-Å filaments from muscle cells. Proc Natl Acad Sci USA 73:4344–4348

    Google Scholar 

  14. Lazarides E, Granger BL, Gard DL, O'Connor CM, Breckler J, Price M, Danto SI (1982) Desmin- and vimentin-containing filaments and their role in the assembly of the Z-disk in muscle cells. Cold Spring harbor Symp Quant Biol 46:413–429

    Google Scholar 

  15. MacDonald RD, Engel AG (1969) The cytoplasmic body: another structural anomaly of the Z disk. Acta Neuropathol (Berl) 14:99–107

    Google Scholar 

  16. Nakashima N, Tamura Z, Okamoto S, Goto H (1970) Inclusion bodies in human neuromuscular disorder. Arch Neurol 22:270–278

    Google Scholar 

  17. Osborn M, Goebel HH (1983) The cytoplasmic body in a congenital myopathy can be stained with antibodies to desmin, the muscle-specific intermediate filament protein. Acta Neuropathol (Berl) 62:149–152

    Google Scholar 

  18. Patel H, Berry K, MacLeod P, Dunn HG (1983) Cytoplasmic body myopathy. Report on a family and review of the literature. J Neuro Sci 60:281–292

    Google Scholar 

  19. Pelissier JF, Pouget J, Charpin C, Figarella D (1989) Myopathy associated with desmin intermediate filaments. J Neurol Sci 89:49–61

    Google Scholar 

  20. Schröder JM (1982) Pathologie der Muskulatur. Springer, Berlin Heidelberg New York, pp 141–146

    Google Scholar 

  21. Schröder JM, Adams RD (1968) The ultrastructural morphology of the muscle fiber in myotonic dystrophy. Acta Neuropathol (Berl) 10:218–241

    Google Scholar 

  22. Sternberger LA (1986) Immunocytochemistry, 2nd edn. J Wiley & Sons, New York

    Google Scholar 

  23. Stoeckel ME, Osborn M, Porte A, Sacrez A, Batzenschläger A, Weber K (1981) An unusual familial cardiomyopathy characterized by aberrant accumulation of desmin-type intermediate filaments. Virchows Arch [A] 393:53–60

    Google Scholar 

  24. Thornell LE, Edström L, Eriksson A, Henriksson KG, Augqvist KA (1980) The distribution of intermediate protein (skeletin) in normal and diseased human skeletal muscle. An immunohistochemical and electron-microscope study. J Neurol Sci 47:153–170

    Google Scholar 

  25. Wolburg H, Schlote W, Langohr HD, Pfeiffer J, Reiher KH, Heckl RW (1982) Slowly progressive congenital myopathy with cytoplasmic bodies — report of two cases and a review of the literature. Clin Neuropathol 1:55–66

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Recipient of a grant from the Deutsche Forschungsgemeinschaft.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schröder, J.M., Sommer, C. & Schmidt, B. Desmin and actin associated with cytoplasmic bodies in skeletal muscle fibers: immunocytochemical and fine structural studies, with a note on unusual 18- to 20-nm filaments. Acta Neuropathol 80, 406–414 (1990). https://doi.org/10.1007/BF00307695

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00307695

Key words

Navigation