Summary
The homozygous wobbler (wr) mouse mutant manifests shortly after birth a form of progressive neural atrophy characterized pathologically by vacuolization and degeneration of neurons of the brainstem and ventral horn of the spinal cord. Ultrastructural features include the presence of autophagic vacuoles containing tubular structures indistinguishable in appearance from neurotubules. The present study correlates certain of these histological features with the characteristics of axoplasmic flow in the homozygous 5–8 week old (wr) mouse.
Slow phase axoplasmic flow was found to be identical in both (wr) mice and normal littermate groups and was estimated to be 1–2 mm per day. However, the homozygous (wr) mouse consistently demonstrated more label in the proximal segments at 7 and 10 days suggesting the presence of an additional population of neurons with impaired flow but with a relatively intact protein synthetic mechanism. This is accompanied by histologic evidence for the elaboration of abnormal neurotubular protein under genetic control. It is hypothesized that the neurotubular transport system underlying the slow phase of axoplasmic flow is thus rendered defective resulting in impairment in axonal continuity and the progressive histologic picture so described.
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Supported in part by grants: CA 08543 from the National Cancer Institute, General Research Support Grant Number 5409, National Institutes of Health. The Jack N. Meeks Research Fund. CA 11224 and GM 1052, National Institutes of Health.
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Bird, M.T., Shuttleworth, E., Koestner, A. et al. The wobbler mouse mutant: An animal model of hereditary motor system disease. Acta Neuropathol 19, 39–50 (1971). https://doi.org/10.1007/BF00690953
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DOI: https://doi.org/10.1007/BF00690953