{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T20:06:41Z","timestamp":1790798801889,"version":"4.1.0"},"reference-count":85,"publisher":"Portland Press Ltd.","issue":"2","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,4,17]]},"abstract":"<jats:p>Nesprins (nuclear envelope spectrin repeat proteins) are multi-isomeric scaffolding proteins. Nesprin-1 and -2 are highly expressed in skeletal and cardiac muscles and together with SUN (Sad1p\/UNC84) domain-containing proteins form the LInker of Nucleoskeleton and Cytoskeleton (LINC) complex at the nuclear envelope in association with lamin A\/C and emerin. Mutations in nesprin-1\/2 have been found in patients with autosomal dominant Emery\u2013Dreifuss muscular dystrophy (EDMD) as well as dilated cardiomyopathy (DCM). Several lines of evidence indicate that compromised LINC complex function is the critical step leading to muscle disease. Here, we review recent advances in our understanding of the functions of nesprin-1\/2 in the LINC complex and mechanistic insights into how mutations in nesprin-1\/2 lead to nesprin-related muscle diseases, in particular DCM and EDMD.<\/jats:p>","DOI":"10.1042\/bst20170149","type":"journal-article","created":{"date-parts":[[2018,2,28]],"date-time":"2018-02-28T01:35:12Z","timestamp":1519781712000},"page":"311-320","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["Nesprin-1\/2: roles in nuclear envelope organisation, myogenesis and muscle disease"],"prefix":"10.1042","volume":"46","author":[{"given":"Can","family":"Zhou","sequence":"first","affiliation":[{"name":"Department of Cardiology, West China Hospital of Sichuan University, Chengdu 610041, China"},{"name":"King's College London British Heart Foundation Centre of Research Excellence, Cardiovascular Division, James Black Centre, 125 Coldharbour Lane, London SE5 9NU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Rao","sequence":"additional","affiliation":[{"name":"Department of Cardiology, West China Hospital of Sichuan University, Chengdu 610041, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine M.","family":"Shanahan","sequence":"additional","affiliation":[{"name":"King's College London British Heart Foundation Centre of Research Excellence, Cardiovascular Division, James Black Centre, 125 Coldharbour Lane, London SE5 9NU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiuping","family":"Zhang","sequence":"additional","affiliation":[{"name":"King's College London British Heart Foundation Centre of Research Excellence, Cardiovascular Division, James Black Centre, 125 Coldharbour Lane, London SE5 9NU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2018,2,27]]},"reference":[{"issue":"Pt 24","key":"2021111818064708900_BST-2017-0149CC1","doi-asserted-by":"crossref","first-page":"4485","DOI":"10.1242\/jcs.114.24.4485","article-title":"Nesprins: a novel family of spectrin-repeat-containing proteins that localize to the nuclear membrane in multiple tissues","volume":"114","author":"Zhang","year":"2001","journal-title":"J. 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