Age‐dependent neuromuscular impairment in prion protein knockout mice. Issue 2 (26th November 2015)

• Record Type: Journal Article
• Authors: Massimino, Maria Lina; Peggion, Caterina; Loro, Federica; Stella, Roberto; Megighian, Aram; Scorzeto, Michele; Blaauw, Bert; Toniolo, Luana; Sorgato, Maria Catia; Reggiani, Carlo; Bertoli, Alessandro
• Is Part Of: Muscle & nerve. Volume 53:Issue 2(2016)
• Page Start: 269
• Page End: 279
• Publication Date: 2015-11-26
• Subjects: demyelination -- prion protein -- sciatic nerve -- skeletal muscle -- treadmill; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.24708
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction : The cellular prion protein (PrP C ) 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 PrP C is still ambiguous. Evidence has suggested that PrP C is involved in different cellular functions, including peripheral nerve integrity and skeletal muscle physiology . Methods : We analyzed the age‐dependent influence of PrP C 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, PrP C . 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