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Accelerated ageing syndromes such as Hutchinson\u2013Gilford Progerin Syndrome, caused by mutations in genes encoding nuclear envelope proteins, have been extensively studied to aid our understanding of the normal biological ageing process. Like several other pathologies associated with genetic defects to nuclear envelope proteins including Emery\u2013Dreifuss muscular dystrophy, Limb\u2013Girdle muscular dystrophy and congenital muscular dystrophy, these disorders can lead to severe muscle dysfunction. Here, we first describe the structure and function of nuclear envelope proteins, and then review the mechanisms by which mutations in genes encoding nuclear envelope proteins induce premature ageing diseases and muscle pathologies. In doing so, we highlight the potential importance of such genes in processes leading to skeletal muscle weakness in old age.<\/jats:p>","DOI":"10.1042\/cs20190066","type":"journal-article","created":{"date-parts":[[2020,8,26]],"date-time":"2020-08-26T07:34:48Z","timestamp":1598427288000},"page":"2177-2187","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Using nuclear envelope mutations to explore age-related skeletal muscle weakness"],"prefix":"10.1042","volume":"134","author":[{"given":"Edmund","family":"Battey","sequence":"first","affiliation":[{"name":"Centre of Human and Applied Physiological Sciences, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, King\u2019s College London, London, SE1 1UL, U.K."},{"name":"British Heart Foundation Centre of Excellence, School of Cardiovascular Medicine and Sciences, King\u2019s College London, London, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew\u00a0J.","family":"Stroud","sequence":"additional","affiliation":[{"name":"British Heart Foundation Centre of Excellence, School of Cardiovascular Medicine and Sciences, King\u2019s College London, London, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6358-2920","authenticated-orcid":false,"given":"Julien","family":"Ochala","sequence":"additional","affiliation":[{"name":"Centre of Human and Applied Physiological Sciences, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, King\u2019s College London, London, SE1 1UL, U.K."},{"name":"Randall Centre for Cell and Molecular Biophysics, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, Guy's Campus, King's College London, SE1 1UL, U.K."},{"name":"Department of Biomedical Sciences, University of Copenhagen, Blegdamsvej 3, 2200 Copenhagen N, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2020,8,26]]},"reference":[{"key":"2021113011152686700_B1","article-title":"World population prospects","author":"United Nations","year":"2019"},{"key":"2021113011152686700_B2","article-title":"Life Expectancy \u2014 Our World in Data","author":"Roser","year":"2019"},{"key":"2021113011152686700_B3","doi-asserted-by":"publisher","first-page":"137","DOI":"10.15252\/embr.201439518","article-title":"Living too long","volume":"16","author":"Brown","year":"2015","journal-title":"EMBO Rep."},{"key":"2021113011152686700_B4","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1007\/s00198-015-3236-5","article-title":"Age-associated declines in muscle mass, strength, power, and physical performance: impact on fear of falling and quality of life","volume":"27","author":"Trombetti","year":"2016","journal-title":"Osteoporos. 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