Age‐dependent neuromuscular impairment in prion protein knockout mice |
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| Authors: | Maria Lina Massimino PhD Caterina Peggion PhD Federica Loro BSc Roberto Stella PhD Aram Megighian PhD Michele Scorzeto PhD Bert Blaauw PhD Luana Toniolo PhD Maria Catia Sorgato PhD Carlo Reggiani PhD Alessandro Bertoli PhD |
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| Institution: | 1. CNR Neuroscience Institute, University of Padova;2. Department of Biomedical Sciences, University of Padova, Padova, Italy |
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| Abstract: | Introduction: The cellular prion protein (PrPC) is commonly recognized as the precursor of prions, the infectious agents of the fatal transmissible spongiform encephalopathies, or prion diseases. Despite extensive effort, the physiological role of PrPC is still ambiguous. Evidence has suggested that PrPC is involved in different cellular functions, including peripheral nerve integrity and skeletal muscle physiology. Methods: We analyzed the age‐dependent influence of PrPC on treadmill test–based aerobic exercise capacity and on a series of morphological and metabolic parameters using wild‐type and genetically modified mice of different ages expressing, or knockout (KO) for, PrPC. Results: We found that aged PrP‐KO mice displayed a reduction in treadmill performance compared with PrP‐expressing animals, which was associated with peripheral nerve demyelination and alterations of skeletal muscle fiber type. Conclusion: PrP‐KO mice have an age‐dependent impairment of aerobic performance as a consequence of specific peripheral nerve and muscle alterations. Muscle Nerve 53: 269–279, 2016 |
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| Keywords: | demyelination prion protein sciatic nerve skeletal muscle treadmill |
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