Advertisement

Virchows Archiv B

, 4:21 | Cite as

Mitochondrial ultrastructure with crystalloid inclusions in an unusual type of human myopathy

  • J. P. M. Schellens
  • E. Ossentjuk
Article
  • 22 Downloads

Summary

A patient is described who suffered from an unusual type of myopathy characterized biochemically by mitochondria in a state of loosely coupled oxidative phosphorylation. The unusual fatigability with exertion was accompanied by pain; wasting of muscle was absent. Cardiac involvement was probable.

Histochemistry pointed to a high activity of oxidative enzymes and mitochondrial ade-nosinetriphosphatase in the type I fibres.

Ultrastructurally the mitochondria showed a number of unusual features; the most striking was the presence of highly regular crystal-like inclusions which were composed of particles with a periodicity in the order of 80 Å and which probably were situated between the inner and outer mitochondrial membranes.

Further mitochondrial features were: unusual sizes and shapes, an inhomogeneous matrix containing large electron dense inclusions, and particulate dots which seemed to lie within the cristae.

Some myofibrils were branched. Accumulations of electron dense material, similar to and continuous with Z-lines were observed.

Keywords

Myopathy Electron Dense Material Muscle Mitochondrion Cross Striation Electron Dense Inclusion 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Mitochondrien-Feinstruktur mit kristallinen Einschlüssen in einer ungewöhnlichen Form von menschlicher Myopathie

Zusammenfassung

Bericht über einen Patienten, der an einer Myopathie litt, wobei die Mitochondrien eine aufgelockerte Kupplung der oxydativen Phosphorylierung zeigten. Der Patient war bald erschöpft und erlitt Schmerzen nach Anstrengung. Muskelatrophie fand nicht statt. Beteiligung des Herzens schien wahrscheinlich. Histochemische Untersuchungen deuteten auf eine hohe Aktivität von oxydativen Fermenten und Adenosintriphosphatase in den Mitochondrien der Typ I-Fasern.

Die Feinstruktur der Mitochondrien wies mehrere eigentümliche Veränderungen auf. Am bemerkenswertesten waren sehr regelmäßig geformte Einschlüsse, die aus Körperchen mit einer Periodizität von etwa 80 Å gebaut waren und die wahrscheinlich zwischen den inneren und äußeren Mitochondrienmembranen lagen. Einige Mitochondrien hatten unge-wöhnliche Formen und Größen. Andere besaßen ein inhomogenes Matrix mit großen elektronen-dichten Einschlüssen oder eine feinkörnige Substanz, die innerhalb der Cristae abgelagert war. Einige Myofibrillen waren verästelt. Anhäufungen von elektronendichter Substanz waren vorhanden, die ähnlich zu und kontinuierlich mit Z-Linien waren.

Literature

  1. Chou, S. M.: “Megaoonial” mitochondria observed in a case of chronic polymyositis. Acta neuropath. (Berl.)12, 68–89 (1969).CrossRefGoogle Scholar
  2. D’Agostino, A. N., F. A. Ziter, M. L. Rallison, and P. F. Bray: Familial myopathy with abnormal muscle mitochondria. Arch. Neurol. (Chic.)18, 388–401 (1968).Google Scholar
  3. Hülsmann, W. C., J. Bethlem, A. E. F. H. Meijer, P. Fleury, and J. P. M. Schellens: Myopathy with abnormal structure and function of muscle mitochondria. J. Neurol. Neurosurg. Psychiat.30, 519–525 (1967).PubMedCrossRefGoogle Scholar
  4. Luft, R., D. Ikkos, G. Palmieri, L. Ernster, and B. Afzelius: A case of severe hypermetabolism of nonthyroid origin with a defect in the maintenance of mitoehondrial respiratory, a correlated clinical, biochemical and morphological study. J. clin. Invest.41, 1776–1804 (1962).PubMedCrossRefGoogle Scholar
  5. Norris, F. H., and B. J. Panner: Hypothyroid myopathy. Arch. Neurol. (Chic.)14, 574–589 (1966).Google Scholar
  6. Price, H. M., G. B. Gordon, Th. L. Munsat, and C. M. Pearson: Myopathy with atypical mitochondria in type I skeletal muscle fibres. J. Neuropathol. exp. Neurol.26, 475–497 (1967).PubMedCrossRefGoogle Scholar
  7. Shafiq, S. A., M. Gorycki, L. Goldstone, and A. T. Milhorat: Fine structure of fibre types in normal human muscle. Anat. Rec.156, 283–302 (1966).CrossRefGoogle Scholar
  8. Shy, G. M., N. K. Gonatas, and M. Perez: Two childhood myopathies with abnormal mitochondria. I. Megaconial myopathy. II. Pleoconial myopathy. Brain89, 133–158 (1966).PubMedCrossRefGoogle Scholar
  9. Wijngaarden, G. K. van, J. Bethlem, A. E. F. H. Meijer, W. Ch. Hülsmann, and C. A. Feltkamp: Skeletal muscle disease with abnormal mitochondria. Brain90, 577–592 (1967).PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1969

Authors and Affiliations

  • J. P. M. Schellens
    • 1
  • E. Ossentjuk
    • 2
  1. 1.Histologie LaboratoryUniversity of AmsterdamAmsterdamThe Netherlands
  2. 2.Neurology ClinicState University of UtrechtNetherlands

Personalised recommendations