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Zur Ultrastruktur des Speichermaterials von spätinfantiler amaurotischer Idiotie

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Zusammenfassung

Im neuralen Speichermaterial eines Falles von myoklonischer Form der spätinfantilen amaurotischen Idiotie, konnten polymorphe Ablagerungen nachgewiesen werden. Membrankörper (oligo- und unizentrisch geordnete) sind Ausgangsformen der verschiedenen Depositformen. Für die überwiegende Depositform konnte nachgewiesen werden, daß die verschieden-strukturierten Ablagerungen vorwiegend aus oligozentrischen Membrankörpern entstehen und die morphologischen Manifestation verschiedener Stufen einer kontinuierlich verfolgbaren Stadienfolge darstellen, die schließlich zur einem lipopigmentartigen Deposittyp führt. Diese Transformationsreihe ist der Ausdruck eines krankheitsunspezifischen Strukturierungsprozesses, der im Auf- und Abbau von Membranen und Membrankörpern besteht und sich am krankheitsspezifischen Produkt — den deponierten Lipoidverbindungen — abspielt. Die Möglichkeit einer initialen Beteiligung der Mitochondrien an der Formierung der oligozentrischen Membrankörper wird erörtert.

Summary

By way of summary we say that in the material obtained in a case of myoclonic form of late-infantile amaurotic idiocy polymorphous deposits were found. Membrane bodies (oligo- and unicentrically arranged) are the basic structures of the different forms of deposits. The greater part of these different kinds of deposits show that the deposits varying in structure originate above all from oligocentric membrane bodies and are the morphological manifestations of various stages of a continuous process with clearly discernible stages of development, terminating in a lipopigment-like type of deposits. This transformation series corresponds to a structural process unspecific to the disease, which consists in the composition and degeneration of membranes and membrane bodies and takes place in the product specific to the disease namely the deposited lipid compounds. The possible participation, at an early stage, of mitochondria in the formation of membrane bodies could be shown.

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Literatur

  • Bielschowsky, M.: Über spätinfantile amaurotische Idiotie mit Kleinhirnsymptomen. Dtsch. Z. Nervenheilk.50, 7–29 (1914)

    Google Scholar 

  • Cotte, G.: Quelques problems posés par l'ultrastructure des lipides de la cortico-surrénale. J. Ultrastruct. Res.3, 186–209 (1959).

    Google Scholar 

  • Daems, W. T., andT. H. van Rjissel: Fine structure of the peribiliary dense bodies in mouse liver tissue. J. Ultrastruct. Res.5, 263–290 (1961).

    Google Scholar 

  • Diezel, P. B.: Die Stoffwechselstörung der Sphingolipoide. Berlin, Göttingen, Heidelberg: Springer 1957.

    Google Scholar 

  • Escola Pico, J.: Über die Ultrastruktur der Speichersubstanzen bei Spätfällen von familiärer amaurotischer Idiotie. Acta neuropath. (Berl.)3, 309–318 (1964).

    Google Scholar 

  • —: Über die Feinstruktur der Speichersubstanzen bei der infantilen amaurotischen Idiotie. Acta neuropath. (Berl.)3, 289–294 (1964).

    Google Scholar 

  • Finean, J. B.: Electron microscope and X Ray diffraction of a saturated synthetic phospholipid. J. biophys. biochem. Cytol.6, 123–124 (1959).

    Google Scholar 

  • Geren, B. B., andF. O. Schmitt: Structure on nerve sheat in relations to lipid and lipid-protein layers. J. appl. Physiol.24, 1421 (1953).

    Google Scholar 

  • Gonatas, N., andJ. Gonatas: Ultrastructural and biochemical observations of systemic late infantile lipidosis and its relationship to Tay-Sachs disease and gargoylism. J. Neuropath. exp. Neurol.24, 318–340 (1965).

    Google Scholar 

  • Gonatas, N. K., R. D. Terry, R. Winkler, S. R. Korey, C. J. Gomez, andA. Stein: A case of juvenile lipidosis. The significance of electron microscopic and biochemical observations of a cerebral biopsy. J. Neuropath. exp. Neurol.22, 557–580 (1963).

    Google Scholar 

  • Jatzkewitz, H., H. Pilz u.K. Sandhoff: Quantitative Bestimmungen von Gangliosiden und ihren neuraminsäurefreien Derivaten bei infantilen, juvenilen und adulten Formen der amaurotischen Idiotie und einer spätinfantilen biochemischen Sonderform. J. Neurochem.12, 135–144 (1965).

    Google Scholar 

  • Jecequel, A. M.: Dégénérescence myelinique des mitochondries de foie humaine dans un epithelioma. J. Ultrastruct. Res.3, 210–215 (1959).

    Google Scholar 

  • Jervis, G. A.: Juvenile amaurotic idiocy. J. Dis. Child.97, 663–667 (1959).

    Google Scholar 

  • Karrer, H. E.: Electron microscopic observations on chick embryo liver. J. Ultrastruct. Res.5, 116–141 (1961).

    Google Scholar 

  • —: The ultrastructure of the lung. The alveolar macrophage. J. biophys. biochem. Cytol.4, 693–700 (1958).

    Google Scholar 

  • Klenk, E.: Über die Ganglioside des Gehirns bei infantiler amaurotischer Idiotie. Ber. dtsch. chem. Ges.75, 1632 (1942).

    Google Scholar 

  • —, andK. Sandhoff: On a biochemically special form of infantile amaurotic idiocy. Biochem. biophys. Acta (Amst.)70, 354–356 (1963).

    Google Scholar 

  • Luzatti, V., andF. Husson: The structure of the lipid-crystalline phase of lipid-water systems. J. Cell Biol.12, 207–219 (1962).

    Google Scholar 

  • Miller, F.: Lipoprotein granules in the cortical collecting tubules of mouse kidney. J. biophys. biochem. Cytol.9, 157–170 (1961).

    Google Scholar 

  • Norman, R. M.: Neuropathology. Ed.J. C. Greenfield. London: Arnold 1958.

    Google Scholar 

  • Policard, A., M. Bessis etJ. M. Breton-Gorius: Structure myélinique observée au microscope electronique sur des coupes de globules rouge en voie de lyse. Exp. Cell Res.13, 184–186 (1957).

    Google Scholar 

  • Revel, J. P., S. Ito, andD. W. Fawcett: Electron micrographs of myelin figures of phospholipid, simulating intracellular membranes. J. biophys. biochem. Cytol.4, 495–498 (1958).

    Google Scholar 

  • Robertis, E., De, andD. Sabatini: Mitochondrial changes in the adrenocortex of the normal hamster. J. biophys. biochem. Cytol.4, 667–670 (1958).

    Google Scholar 

  • Seitelberger, F.: Sonderformen cerebraler Lipoidosen. Proc. IV. Int. Kongr. Neuropath. München 1961 Vol. I, pp. 3–13. Stuttgart: G. Thieme 1962.

    Google Scholar 

  • —, u.K. Nagy: Zur Histopathologie und Klinik der Spätformen von amaurotischer Idiotie. Dtsch. Z. Nervenheilk.177, 577–596 (1958).

    Google Scholar 

  • —,G. Vogel u.H. Stepan: Spatinfantile amaurotische Idiotie. Arch. Psychiat. Nervenkr.196, 154–190 (1957).

    Google Scholar 

  • Stockenius, W.: Morphologische Beobachtungen beim intracellulären. Erythrocytenabbau und der Eisenspeicherung in der Milz des Kaninchens. Klin. Wschr.35, 760–763 (1957).

    Google Scholar 

  • —: The molecular structure of lipid-water systems and the cell membrane models studied with the electron microscope. Symposia of the Int. Soc. for Cell Biology, Vol.I, pp. 349 to 367 (1961).

    Google Scholar 

  • Stockenius, W.: Some electron microscopical observations on liquid-cristalline phase in lipid-water systems. J. Cell Biol.12, 221–229 (1962).

    Google Scholar 

  • Terry, R. D., andS. Korey: Studies in Tay-Sachs disease. V. The membranes of the membranous cytoplasmic bodies. J. Neuropath. exp. Neurol.22, 98–104 (1963)

    Google Scholar 

  • —— andM. Weiss: Electron microscopy of the cerebrum in Tay-Sachs disease. In: Cerebral Sphingolipoidoses, pp. 49–56. New York and London: Acad. Press 1962.

    Google Scholar 

  • —, andM. Weiss: Studies in Tay-Sachs disease. II. Ultrastructure of the cerebrum. J. Neuropath. exp. Neurol.22, 18–55 (1963).

    Google Scholar 

  • Samuels, S., K. Gonatas, andM. Weiss: Chemical and ultrastructural comparison of synthetic and pathologie membrane systems. J. Cell. Biol.21, 148–152 (1964).

    Google Scholar 

  • —,S. Korey, J. Gonatas, R. D. Terry, andM. Weiss: Studies in Tay-Sachs disease. IV. Membranous cytoplasmatic bodies. a) Biochemistry. J. Neuropath. exp. Neurol.22, 81–97 (1963).

    Google Scholar 

  • —,S. Korey, J. Gonatas, R. D. Terry, andM. Weiss: Studies in Tay-Sachs disease. IV. Membranous cytoplasmatic bodies. b) Ultrastructure. J. Neuropath. exp. Neurol.22, 81–97 (1963).

    Google Scholar 

  • Thoenes, W.: Fine structure of lipid granules in proximal tubule cells of mouse kidney. J. Cell Biol.12, 433–437 (1962).

    Google Scholar 

  • Wallace, B. J., S. S. Lazarus, L. Schneck, andB. V. Volk: Some biochemical, histochemical und fine structural aspects of a case of late infantile Tay-Sachs disease (Jansky-Bielschowsky Typ). J. Neuropath. exp. Neurol.24, 169–170 (1965).

    Google Scholar 

  • Wyburn-Mason, R.: On some anomalous forms of amaurotic idiocy and their bearing on the relationship of the various types. Brit. J. Ophthal.27, 145–173 (1943).

    Google Scholar 

  • Zeman, W., andSh. Donahue: Fine structure of lipid bodies in juvenile idiocy. Acta neuropath. (Berl.)3, 144–149 (1963).

    Google Scholar 

  • —, andJ. Hoffman: Juvenile and late form of amaurotic idiocy in one family. J. Neurol. Neurosurg. Psychiat.25, 352–362 (1961).

    Google Scholar 

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Authors and Affiliations

  1. Neurologisches Institut der Universität Wien, Schwarzspanierstr. 17, Wien, Österreich

    E. Sluga & Th. Majdetzki

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  1. E. Sluga
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  2. Th. Majdetzki
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Nach einer Mitteilung vor der Österr. Arbeitsgemeinschaft für Neuropathologie am 6. November 1965.

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Sluga, E., Majdetzki, T. Zur Ultrastruktur des Speichermaterials von spätinfantiler amaurotischer Idiotie. Acta Neuropathol 9, 254–272 (1967). https://doi.org/10.1007/BF00687935

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  • DOI: https://doi.org/10.1007/BF00687935

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