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Etude histochimique des corps de Lafora

Histochemical study of Lafora bodies

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Summary

The so-called Lafora's bodies were studied by histochemical techniques for mucopolysaccharides and glycoproteins.

The results do not confirm the hypothesis according to which glycogen or acid mucopolysaccharides are present in Lafora's bodies. However, the existence of the following fractions in these inclusions could be ascertained:

  1. 1.

    A carbohydrate moiety, characterized by

    1. a)

      the presence of periodate reactive vic-glycols adjacent to acidic groups, carboxylic and possibly phosphates;

    2. b)

      its content in hexoses.

  2. 2.

    A proteic moiety which appears to be desintegrated and separated from the carbohydrate fraction by the action of papain. This enzyme indeed alters the alcianophily (at pH 2.5) in the core of complex Lafora's bodies. This result suggests that the enzyme might release strong acidic groups at the junction between the two above-mentioned moieties.

The question still remains whether the Lafora's bodies contain only one complex molecule with various chemical properties or two (or more) different molecules.

Résumé

Des techniques histochimiques modernes des mucopolysaccharides et des glycoproteines ont été appliquées, à l'étude des corps de Lafora, observées dans l'épilepsie-myoclonie.

Les résultats obtenus ne plaident pas en faveur de la présence dans les corps de Lafora de glycogène ou de mucopolysaccharides acides, mais permettent d'établir l'existence au sain de ces inclusions, de molécules complexes comprenant

  1. 1.

    Une fraction sucrée caractérisée d'une part par la présence de groupes vic-glycols oxydables par l'acide periodique et situés à proximité de groupes acides carboxyles et éventuellement phosphates, d'autre part par son contenu en hexoses.

  2. 2.

    Une fraction protéique qui se laisse apparemment digérer et cliver de la fraction sucrée par la papaïne; l'enzyme altère en effet l'alcianophilie à pH 2,5 du centre des corps complexes comme si elle libérait des groupes acides forts situés éventuellement à la jonction des deux fractions sus-décrites.

Ces observations ne permettent pas d'établir s'il existe au sein des corps de Lafora un seul corps complexe aux propriétés chimiques diverses ou deux ou plusieurs composés chimiques différents.

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Abbreviations

PAS:

Periodic acid Schiff

PA-paraD:

Periodic acid paradiamine

PA-métaD:

Periodic acid métadiamine

BA:

Bleu Alcian

BT:

Bleu de Toluidine

AF:

Aldéhyde Fuchsine

HID:

High Iron Diamines

Chr 2R:

Chromotrope 2R

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  1. Laboratoire d'Anatomie Pathologique Faculté de Médecine, Université Libre de Bruxelles, 97, Rue aux Laines, Bruxelles, Belgique

    M. Dubois-Dalcq (Aspirant F. N. R. S.)

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Dubois-Dalcq, M. Etude histochimique des corps de Lafora. Acta Neuropathol 12, 205–217 (1969). https://doi.org/10.1007/BF00687645

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