Summary
The enzyme histochemistry of the cerebellum, pons, medulla and spinal cord of rabbits with hereditary ataxia is reported.
Nerve cells, mainly within the vestibular and cochlear nuclei and the dentate nuclei showed an increase in glucose 6-phosphate dehydrogenase activity and a decrease in enzymes of Krebs (tricarboxylic acid) cycle suggesting that production of energy yielding metabolites may have been impaired. The decrease in activity of the Krebs cycle enzymes and of monoamine oxidase in the neuropil of affected nuclei suggested that an alteration in metabolism within the neuropil was an important factor.
Glycogen deposits were more prominent in the affected cerebellar nuclei and tracts than in the brainstem, but the enzyme glycogen-phosphorylase was generally normal.
There was no evidence of massive gliosis but the intense oxidative enzyme activities within swollen hyperactive astrocytes in affected nuclei and tracts indicated increased metabolism in these cells.
Zusammenfassung
Bericht über enzymhistochemische Untersuchungen von Kleinhirn, Brücke, Medulla und Rückenmark von Ratten mit hereditärer Ataxie.
Die Nervenzellen, vorwiegend in den Vestibularis- und Cochleariskernen sowie im Zahnkern, zeigten eine Zunahme der Glucose-6-Phosphat-Dehydrogenase-Aktivität und Abnahme von Enzymen des Krebs(Tricarbonsäure)-Cyclus, was auf eine Störung der Produktion energiegebundener Metaboliten hinweist. Die Abnahme der Enzyme des Krebs-Cyclus und der Monoaminooxydase im Neuropil der betroffenen Kerne weist darauf hin, daß eine Störung des Stoffwechsels im Neuropil einen wesentlichen Faktor darstellt.
Glykogenablagerungen fanden sich stärker in den betroffenen Kleinhirnkernen und-bahnen als im Hirnstamm, während die Glykogen-Phosphorylase normale Aktivität zeigte.
Eine massive Gliose lag nicht vor, doch fand sich eine starke Aktivität oxydativer Enzyme in geschwollenen Astrocyten in den betroffenen Kernen und Bahnen, die auf einen gesteigerten Stoffwechsel in diesen Zellen hinweist.
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This work was supported by financial assistance from the Action for the Crippled Child Fund. Mr. J. Mills, Department of Neuropathology is kindly thanked for executing the photographic work. The hereditary ataxic rabbits were supplied through the courtesy of Drs. Paul S. Sawin and Richard R. Fox, Jackson Memorial Laboratory, Bar Harbor, Maine. U.S.A.
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Robinson, N. Enzyme changes in the hereditary ataxic rabbit. Acta Neuropathol 14, 326–337 (1970). https://doi.org/10.1007/BF00685788
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DOI: https://doi.org/10.1007/BF00685788