title: Age-dependent neuromuscular impairment in prion protein knockout mice.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  PrP-KO mice have an age-dependent impairment of aerobic performance as a consequence of specific peripheral nerve and muscle alterations.
authors.affiliation: CNR Neuroscience Institute, University of Padova.
authors.forename: Maria Lina
authors.initials: ML
authors.lastname: Massimino
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Caterina
authors.initials: C
authors.lastname: Peggion
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Loro
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Stella
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Aram
authors.initials: A
authors.lastname: Megighian
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Scorzeto
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Bert
authors.initials: B
authors.lastname: Blaauw
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Luana
authors.initials: L
authors.lastname: Toniolo
authors.affiliation: CNR Neuroscience Institute, University of Padova.|Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Maria Catia
authors.initials: MC
authors.lastname: Sorgato
authors.affiliation: CNR Neuroscience Institute, University of Padova.|Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Reggiani
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via U. Bassi 58/B, 35131, Padova, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Bertoli
mesh_terms: D000200:Action Potentials / Q000235:genetics; D000251:Adenosine Triphosphatases / Q000378:metabolism; D000375:Aging; D000818:Animals; D002950:Citrate (si)-Synthase / Q000378:metabolism; D004195:Disease Models, Animal; D005080:Exercise Test; D005786:Gene Expression Regulation / Q000235:genetics; D019344:Lactic Acid / Q000097:blood; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009043:Motor Activity / Q000235:genetics; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology; D053580:Muscle Strength / Q000235:genetics; D018482:Muscle, Skeletal / Q000503:physiopathology; D018995:Myosin Heavy Chains / Q000378:metabolism; D009431:Neural Conduction / Q000235:genetics; D009468:Neuromuscular Diseases / Q000097:blood / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D011328:Prions / Q000235:genetics / Q000378:metabolism*; D012584:Sciatic Nerve / Q000473:pathology; D013385:Succinate Dehydrogenase / Q000378:metabolism
chemical_list: D011328:Prions; D019344:Lactic Acid; D013385:Succinate Dehydrogenase; D002950:Citrate (si)-Synthase; D000251:Adenosine Triphosphatases; D018995:Myosin Heavy Chains
keywords: demyelination; prion protein; sciatic nerve; skeletal muscle; treadmill