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In addition, nonneuronal cells can also be affected as a consequence of primary degeneration or due to neuronal dysfunction. Skeletal muscle is a primary site of toxicity of polyglutamine-expanded androgen receptor, but it is also affected in other polyglutamine diseases, more likely due to neuronal dysfunction and death. Nonetheless, pathological processes occurring in skeletal muscle atrophy impact the entire body metabolism, thus actively contributing to the inexorable progression towards the late and final stages of disease. Skeletal muscle atrophy is well recapitulated in animal models of polyglutamine disease. In this review, we discuss the impact and relevance of skeletal muscle in patients affected by polyglutamine diseases and we review evidence obtained in animal models and patient-derived cells modeling skeletal muscle.<\/jats:p>","DOI":"10.3390\/cells11132105","type":"journal-article","created":{"date-parts":[[2022,7,3]],"date-time":"2022-07-03T22:50:46Z","timestamp":1656888646000},"page":"2105","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Skeletal Muscle Pathogenesis in Polyglutamine Diseases"],"prefix":"10.3390","volume":"11","author":[{"given":"Caterina","family":"Marchioretti","sequence":"first","affiliation":[{"name":"Department of Biomedical Sciences (DBS), University of Padova, 35131 Padova, Italy"},{"name":"Veneto Institute of Molecular Medicine (VIMM), 35129 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Zuccaro","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences (DBS), University of Padova, 35131 Padova, Italy"},{"name":"Veneto Institute of Molecular Medicine (VIMM), 35129 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6267-0179","authenticated-orcid":false,"given":"Udai Bhan","family":"Pandey","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Children\u2019s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA 15100, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4483-386X","authenticated-orcid":false,"given":"Jessica","family":"Rosati","sequence":"additional","affiliation":[{"name":"Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, 71100 Foggia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Basso","sequence":"additional","affiliation":[{"name":"Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, 38100 Trento, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8634-0767","authenticated-orcid":false,"given":"Maria","family":"Pennuto","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences (DBS), University of Padova, 35131 Padova, Italy"},{"name":"Veneto Institute of Molecular Medicine (VIMM), 35129 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/B978-0-444-63233-3.00011-7","article-title":"The CAG-polyglutamine repeat diseases: A clinical, molecular, genetic, and pathophysiologic nosology","volume":"147","author":"Stoyas","year":"2018","journal-title":"Handb. 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