{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T16:17:48Z","timestamp":1774628268333,"version":"3.50.1"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"21","license":[{"start":{"date-parts":[[2019,8,9]],"date-time":"2019-08-09T00:00:00Z","timestamp":1565308800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["094476\/Z\/10\/Z"],"award-info":[{"award-number":["094476\/Z\/10\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Euan MacDonald Centre for Motor Neurone Disease Research"},{"name":"UK SMA Research Consortium"},{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["PG\/16\/68\/31991"],"award-info":[{"award-number":["PG\/16\/68\/31991"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Keele University ACORN"},{"name":"Newlife Charity","award":["SG\/15-16\/11"],"award-info":[{"award-number":["SG\/15-16\/11"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Cardiac pathology is emerging as a prominent systemic feature of spinal muscular atrophy (SMA), but little is known about the underlying molecular pathways. Using quantitative proteomics analysis, we demonstrate widespread molecular defects in heart tissue from the Taiwanese mouse model of severe SMA. We identify increased levels of lamin A\/C as a robust molecular phenotype in the heart of SMA mice and show that lamin A\/C dysregulation is also apparent in SMA patient fibroblast cells and other tissues from SMA mice. Lamin A\/C expression was regulated in vitro by knockdown of the E1 ubiquitination factor ubiquitin-like modifier activating enzyme 1, a key downstream mediator of SMN-dependent disease pathways, converging on \u03b2-catenin signaling. Increased levels of lamin A are known to increase the rigidity of nuclei, inevitably disrupting contractile activity in cardiomyocytes. The increased lamin A\/C levels in the hearts of SMA mice therefore provide a likely mechanism explaining morphological and functional cardiac defects, leading to blood pooling. Therapeutic strategies directed at lamin A\/C may therefore offer a new approach to target cardiac pathology in SMA.<\/jats:p>","DOI":"10.1093\/hmg\/ddz195","type":"journal-article","created":{"date-parts":[[2019,8,5]],"date-time":"2019-08-05T15:10:49Z","timestamp":1565017849000},"page":"3515-3527","source":"Crossref","is-referenced-by-count":12,"title":["Lamin A\/C dysregulation contributes to cardiac pathology in a mouse model of severe spinal muscular atrophy"],"prefix":"10.1093","volume":"28","author":[{"given":"Darija","family":"\u0160olti\u0107","sequence":"first","affiliation":[{"name":"Institute for Science and Technology in Medicine, Keele University, Keele ST5 5BG, UK"},{"name":"Wolfson Centre for Inherited Neuromuscular Disease, RJAH Orthopaedic Hospital, Oswestry SY10 7AG, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannah K","family":"Shorrock","sequence":"additional","affiliation":[{"name":"Edinburgh Medical School: Biomedical Sciences"},{"name":"Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Edinburgh EH8 9XD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hazel","family":"Allardyce","sequence":"additional","affiliation":[{"name":"Institute of Education for Medical and Dental Science, College of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB24 3FX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma L","family":"Wilson","sequence":"additional","affiliation":[{"name":"Chester Medical School, University of Chester, Chester CH1 4BJ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian","family":"Holt","sequence":"additional","affiliation":[{"name":"Wolfson Centre for Inherited Neuromuscular Disease, RJAH Orthopaedic Hospital, Oswestry SY10 7AG, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia A","family":"Synowsky","sequence":"additional","affiliation":[{"name":"BSRC Mass Spectrometry and Proteomics Facility, University of St Andrews, St Andrews KY16 9ST, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sally L","family":"Shirran","sequence":"additional","affiliation":[{"name":"BSRC Mass Spectrometry and Proteomics Facility, University of St Andrews, St Andrews KY16 9ST, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon H","family":"Parson","sequence":"additional","affiliation":[{"name":"Institute of Education for Medical and Dental Science, College of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB24 3FX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0306-5577","authenticated-orcid":false,"given":"Thomas H","family":"Gillingwater","sequence":"additional","affiliation":[{"name":"Edinburgh Medical School: Biomedical Sciences"},{"name":"Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Edinburgh EH8 9XD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heidi R","family":"Fuller","sequence":"additional","affiliation":[{"name":"Institute for Science and Technology in Medicine, Keele University, Keele ST5 5BG, UK"},{"name":"Wolfson Centre for Inherited Neuromuscular Disease, RJAH Orthopaedic Hospital, Oswestry SY10 7AG, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2019,8,9]]},"reference":[{"key":"2019122311002926800_ref1","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1038\/nrneurol.2018.4","article-title":"Advances in therapy for spinal muscular atrophy: promises and challenges","volume":"14","author":"Groen","year":"2018","journal-title":"Nat. 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