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A biochemical and ultrastructural study of liver, muscle, heart and kidney in type II glycogenosis

Biochemische und ultrastrukturelle Untersuchungen von Leber, Muskulatur, Herz und Nieren bei Glykogenosis II

  • Published:
Virchows Archiv B

Zusammenfassung

In der vorliegenden Studie werden biochemische und elektronenmikroskopische Untersuchungen der Leber, der Muskulatur, des Herzens und der Niere in einem Fall von Glykogenose Typ II vorgelegt.

Folgende Befunde wurden erhoben:

  1. 1.

    Im Herzmuskel liegt das Glykogen nicht in membrangebundener Form vor. Das Herzmuskelglykogen unterscheidet sich in seiner Form, der Größe und dem Sedimentationsverhalten vom Muskelglykogen.

  2. 2.

    Das Leberglykogen ist ebenfalls abnorm hinsichtlich der Größe und der Verteilung der Partikel. Es kommen außerdem mehr kleinere und leichtere Partikel vor.

  3. 3.

    Im Nierengewebe findet man eine hohe Aktivität an saurer Maltase, obwohl zahlreiche Lysosomen Glykogen enthalten. Biochemische Untersuchungen weisen auf die Möglichkeit verschiedener Isoenzyme der sauren Nierenmaltase hin.

Summary

The present study deals with ultrastructural findings and biochemical data from liver, muscle, heart and kidney in a case of type II glycogenosis. A detailed physicochemical and ultrastructural study of the glycogen particles isolated from these tissues has also been performed.

The main findings can be summarized as follows:

  1. 1.

    The data obtained from the ultrastructural study of the cardiac tissue show the glycogen is not within bodies surrounded by a membrane. The shape, size and sedimentation pattern of cardiac glycogen are different from those of the muscle.

  2. 2.

    The glycogen isolated from liver is abnormal with respect to size, distribution of the particles and sedimentation pattern: smaller, lighter particles predominate.

  3. 3.

    Kidney tissue possesses a high acid maltase activity, but in spite of this, numerous lysosomes filled with glycogen are visible. Biochemical data suggest the possibility that several allelic forms of kidney acid maltase exist.

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Authors and Affiliations

  1. Istituto di Patologia Generale, Università di Napoli, Naples, Italy

    C. B. Bruni & F. M. Paluello

  2. Istituto di Clinica Medica II, Università di Roma, Rome, Italy

    C. B. Bruni & F. M. Paluello

Authors
  1. C. B. Bruni
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  2. F. M. Paluello
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Additional information

This work was partially supported by Grant N. 115/815/4678 from the Consiglio Nazionale delle Ricerche, Comitato Biologia e Medicina, Italy.

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Bruni, C.B., Paluello, F.M. A biochemical and ultrastructural study of liver, muscle, heart and kidney in type II glycogenosis. Virchows Arch. Abt. B Zellpath. 4, 196–207 (1969). https://doi.org/10.1007/BF02906075

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  • DOI: https://doi.org/10.1007/BF02906075

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