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Decrease of enkephalins in cerebellum during Wobbler mouse motoneuron disease
K K Yung1, F Tang, L L Vacca-Galloway
1Department of Anatomy, Faculty of Medicine, University of Hong Kong.
Abstract:
The Wobbler mouse possesses an inherited motoneuron disease, which expresses itself primarily at cervical spinal levels and in cranial motor nuclei. Cell degeneration is sporatic and negligible in other motor regions of the brain (e.g., cerebellum, corpus striatum). However, enkephalin concentrations are consistently lower in the Wobbler cerebellum throughout the motoneuron disease, whereas substance P concentrations are significantly higher late in the disease compared with the normal phenotype littermates. The data imply that early changes in enkephalin (also shown for leucine enkephalin in the spinal cord and brainstem) may be important to the etiology of the Wobbler disorder. Like the late increase of substance P, this may reflect a yet-to-be described response to parent cell degeneration in the raphe nuclei. TRH remained unchanged in Wobbler cerebellum and corpus striatum, wherein the other peptides studied herein also maintained similar concentrations to the normal phenotype littermates.
Insights
Wobbler mice with inherited motoneuron disease show altered neuropeptide levels. Lower enkephalin and higher substance P in the cerebellum suggest these changes are key to the Wobbler disorder.
Area of Science:
- Neuroscience
- Genetics
- Biochemistry
Background:
- The Wobbler mouse model exhibits an inherited motoneuron disease.
- Disease pathology primarily affects cervical spinal cord and cranial motor nuclei.
- Other brain regions like the cerebellum and corpus striatum show minimal cell degeneration.
Purpose of the Study:
- To investigate neuropeptide concentration changes in the Wobbler mouse model.
- To determine the role of specific peptides in the disease's etiology.
- To compare peptide levels between affected mice and normal littermates.
Main Methods:
- Analysis of neuropeptide concentrations (enkephalin, substance P, TRH) in specific brain regions.
- Comparison of peptide levels in Wobbler mice versus normal littermates.
- Focus on cerebellum, spinal cord, brainstem, and corpus striatum.
Main Results:
- Consistently lower enkephalin concentrations in Wobbler cerebellum.
- Significantly higher substance P concentrations late in the disease in Wobbler mice.
- Enkephalin changes (including leucine enkephalin) observed in spinal cord and brainstem.
- Thyrotropin-releasing hormone (TRH) and other peptides remained unchanged in specific regions.
Conclusions:
- Early alterations in enkephalin may be crucial to the Wobbler disorder's development.
- Increased substance P late in the disease might indicate a response to neuronal degeneration.
- These neuropeptide changes offer insights into the pathogenesis of inherited motoneuron diseases.