Summary
Selected lysosomal hydrolases have been investigated in the trigeminal ganglion of mice afflicted with an hereditary sensory neuropathy (dystonia musculorum). This was done using direct enzyme histochemistry. Correlative electron microscopy was also used to further elucidate perikaryal changes. The earliest observed lesion in the trigeminal ganglion of afflicted mice was numerous axon swellings containing intense lysosomal hydrolase activity. Subsequent to this observation, numerous neurones showed central chromatolysis, eccentric nucleus and increased lysosomal hydrolase activity. As various neurones throughout the ganglion underwent the classical chromatolytic reaction, the Golgi apparatus moved to a juxtanuclear location, and there was a focal juxtanuclear accumulation of lysosomes. During the later stages of the disease, a striking decrease in neuronal hydrolase activity characteristic of neuronal atrophy was observed. These results are consistent with earlier suggestions that loss of sensation in the disease could be due to an interruption of axonal transport in primary sensory neurones.
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This investigation was supported by NIH research grant numbers DE02668 and DE00288 from the National Institute of Dental Research and by NIH grant number RR05333 from the Division of Research Facilities and Resources.
In partial fulfillment of the requirements for the Ph. D.
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Thornburg, L.P., Hanker, J.S. Lysosomal hydrolases in the trigeminal ganglion of mice afflicted with an hereditary sensory neuropathy (Dystonia Musculorum). Acta Neuropathol 32, 91–101 (1975). https://doi.org/10.1007/BF00689563
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DOI: https://doi.org/10.1007/BF00689563