Exercise Training Reverses Skeletal Muscle Atrophy in an Experimental Model of VCP Disease
| Autor: | Virginia Kimonis, Veeral Katheria, Angèle Nalbandian, Katrina J. Llewellyn, Christopher Nguyen, Vincent J. Caiozzo, Mallikarjun Badadani |
|---|---|
| Přispěvatelé: | Pandey, Udai |
| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
Aging
lcsh:Medicine Cell Cycle Proteins Apoptosis Neurodegenerative Muscle hypertrophy Mice Valosin Containing Protein Medicine and Health Sciences 2.1 Biological and endogenous factors Aetiology Muscular dystrophy Amyotrophic lateral sclerosis lcsh:Science Adenosine Triphosphatases Multidisciplinary Skeletal Physical Conditioning Muscle atrophy Mitochondria Muscular Atrophy Phenotype Neurological Muscle medicine.symptom Research Article medicine.medical_specialty General Science & Technology Strength training Rare Diseases Atrophy Physical medicine and rehabilitation Physical Conditioning Animal Internal medicine Genetics Autophagy medicine Animals Muscle Strength Muscle Skeletal Myopathy Animal business.industry lcsh:R Muscle weakness medicine.disease Disease Models Animal Endocrinology Musculoskeletal Disease Models Mutation lcsh:Q business |
| Zdroj: | Nalbandian, Angele; Nguyen, Christopher; Katheria, Veeral; Llewellyn, Katrina J.; Badadani, Mallikarjun; Caiozzo, Vincent; et al.(2013). Exercise Training Reverses Skeletal Muscle Atrophy in an Experimental Model of VCP Disease. PLoS ONE, 8(10). UC Irvine: Institute for Clinical and Translational Science. Retrieved from: http://www.escholarship.org/uc/item/9cz8j395 Nalbandian, A; Nguyen, C; Katheria, V; Llewellyn, KJ; Badadani, M; Caiozzo, V; et al.(2013). Exercise Training Reverses Skeletal Muscle Atrophy in an Experimental Model of VCP Disease. PLoS ONE, 8(10). doi: 10.1371/journal.pone.0076187. UC Irvine: Retrieved from: http://www.escholarship.org/uc/item/3rj4j0wn PLoS ONE, Vol 8, Iss 10, p e76187 (2013) PloS one, vol 8, iss 10 PLoS ONE |
| DOI: | 10.1371/journal.pone.0076187. |
| Popis: | Background:The therapeutic effects of exercise resistance and endurance training in the alleviation of muscle hypertrophy/atrophy should be considered in the management of patients with advanced neuromuscular diseases. Patients with progressive neuromuscular diseases often experience muscle weakness, which negatively impact independence and quality of life levels. Mutations in the valosin containing protein (VCP) gene lead to Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD) and more recently affect 2% of amyotrophic lateral sclerosis (ALS)-diagnosed cases.Methods/Principle Findings:The present investigation was undertaken to examine the effects of uphill and downhill exercise training on muscle histopathology and the autophagy cascade in an experimental VCP mouse model carrying the R155H mutation. Progressive uphill exercise in VCPR155H/+ mice revealed significant improvement in muscle strength and performance by grip strength and Rotarod analyses when compared to the sedentary mice. In contrast, mice exercised to run downhill did not show any significant improvement. Histologically, the uphill exercised VCPR155H/+ mice displayed an improvement in muscle atrophy, and decreased expression levels of ubiquitin, P62/SQSTM1, LC3I/II, and TDP-43 autophagy markers, suggesting an alleviation of disease-induced myopathy phenotypes. There was also an improvement in the Paget-like phenotype.Conclusions:Collectively, our data highlights that uphill exercise training in VCPR155H/+ mice did not have any detrimental value to the function of muscle, and may offer effective therapeutic options for patients with VCP-associated diseases. © 2013 Nalbandian et al. |
| Databáze: | OpenAIRE |
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