{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T21:57:16Z","timestamp":1791496636493,"version":"4.3.4"},"reference-count":45,"publisher":"Elsevier BV","issue":"9","license":[{"start":{"date-parts":[[2017,8,1]],"date-time":"2017-08-01T00:00:00Z","timestamp":1501545600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,8,1]],"date-time":"2017-08-01T00:00:00Z","timestamp":1501545600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2017,8,7]],"date-time":"2017-08-07T00:00:00Z","timestamp":1502064000000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Telethon","award":["GEP12008"],"award-info":[{"award-number":["GEP12008"]}]},{"name":"Telethon","award":["GGP15001"],"award-info":[{"award-number":["GGP15001"]}]},{"name":"Association Fran\u00e7aise contres les Myopathies","award":["15999"],"award-info":[{"award-number":["15999"]}]},{"DOI":"10.13039\/501100004189","name":"Max Planck Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004189","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R01 HL082792"],"award-info":[{"award-number":["R01 HL082792"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"BCRP","award":["BC150580"],"award-info":[{"award-number":["BC150580"]}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cell Reports"],"published-print":{"date-parts":[[2017,8]]},"DOI":"10.1016\/j.celrep.2017.08.018","type":"journal-article","created":{"date-parts":[[2017,8,29]],"date-time":"2017-08-29T12:32:58Z","timestamp":1504009978000},"page":"2100-2115","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":55,"title":["Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function"],"prefix":"10.1016","volume":"20","author":[{"given":"Federica F.","family":"Morelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dineke S.","family":"Verbeek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessika","family":"Bertacchini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Vinet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Mediani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Marmiroli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Cenacchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Milena","family":"Nasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"De Biasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanette F.","family":"Brunsting","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Lammerding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Pegoraro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrado","family":"Angelini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossella","family":"Tupler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Alberti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serena","family":"Carra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.celrep.2017.08.018_bib1","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/j.tcb.2016.03.004","article-title":"Phase separation: linking cellular compartmentalization to disease","volume":"26","author":"Aguzzi","year":"2016","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib2","doi-asserted-by":"crossref","first-page":"15041","DOI":"10.1038\/celldisc.2015.41","article-title":"Temporal activation of XRCC1-mediated DNA repair is essential for muscle differentiation","volume":"2","author":"Al-Khalaf","year":"2016","journal-title":"Cell Discov."},{"key":"10.1016\/j.celrep.2017.08.018_bib3","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1002\/bies.201600042","article-title":"Are aberrant phase transitions a driver of cellular aging?","volume":"38","author":"Alberti","year":"2016","journal-title":"BioEssays"},{"key":"10.1016\/j.celrep.2017.08.018_bib4","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1083\/jcb.200904124","article-title":"Role of A-type lamins in signaling, transcription, and chromatin organization","volume":"187","author":"Andr\u00e9s","year":"2009","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib5","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1083\/jcb.132.4.657","article-title":"Myogenin expression, cell cycle withdrawal, and phenotypic differentiation are temporally separable events that precede cell fusion upon myogenesis","volume":"132","author":"Andr\u00e9s","year":"1996","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib6","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/nrm.2017.7","article-title":"Biomolecular condensates: organizers of cellular biochemistry","volume":"18","author":"Banani","year":"2017","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib7","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1016\/j.biocel.2012.03.011","article-title":"The family of mammalian small heat shock proteins (HSPBs): implications in protein deposit diseases and motor neuropathies","volume":"44","author":"Boncoraglio","year":"2012","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib8","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.yexcr.2006.10.004","article-title":"Recruitment of phosphorylated small heat shock protein Hsp27 to nuclear speckles without stress","volume":"313","author":"Bryantsev","year":"2007","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.celrep.2017.08.018_bib9","doi-asserted-by":"crossref","first-page":"3628","DOI":"10.1016\/j.yexcr.2008.09.026","article-title":"Prelamin A is involved in early steps of muscle differentiation","volume":"314","author":"Capanni","year":"2008","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.celrep.2017.08.018_bib10","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.tcb.2013.11.004","article-title":"Broken nuclei--lamins, nuclear mechanics, and disease","volume":"24","author":"Davidson","year":"2014","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib11","doi-asserted-by":"crossref","first-page":"4","DOI":"10.4161\/nucl.13723","article-title":"Investigating the purpose of prelamin A processing","volume":"2","author":"Davies","year":"2011","journal-title":"Nucleus"},{"key":"10.1016\/j.celrep.2017.08.018_bib12","doi-asserted-by":"crossref","first-page":"a000547","DOI":"10.1101\/cshperspect.a000547","article-title":"Nuclear lamins","volume":"2","author":"Dechat","year":"2010","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib13","doi-asserted-by":"crossref","first-page":"1022","DOI":"10.1016\/j.jmb.2009.08.052","article-title":"The small heat-shock proteins HSPB2 and HSPB3 form well-defined heterooligomers in a unique 3 to 1 subunit ratio","volume":"393","author":"den Engelsman","year":"2009","journal-title":"J.\u00a0Mol. Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib14","doi-asserted-by":"crossref","first-page":"11025","DOI":"10.1073\/pnas.162172899","article-title":"Caspase 3 activity is required for skeletal muscle differentiation","volume":"99","author":"Fernando","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2017.08.018_bib15","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1016\/j.molcel.2016.07.021","article-title":"A surveillance function of the HSPB8-BAG3-HSP70 chaperone complex ensures stress granule integrity and dynamism","volume":"63","author":"Ganassi","year":"2016","journal-title":"Mol. Cell"},{"key":"10.1016\/j.celrep.2017.08.018_bib16","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1186\/1471-2121-5-46","article-title":"Altered protein dynamics of disease-associated lamin A mutants","volume":"5","author":"Gilchrist","year":"2004","journal-title":"BMC Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib17","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1038\/emboj.2009.196","article-title":"Novel roles for A-type lamins in telomere biology and the DNA damage response pathway","volume":"28","author":"Gonzalez-Suarez","year":"2009","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2017.08.018_bib18","doi-asserted-by":"crossref","first-page":"e0133994","DOI":"10.1371\/journal.pone.0133994","article-title":"Characterization of the cardiac overexpression of HSPB2 reveals mitochondrial and myogenic roles supported by a cardiac HspB2 interactome","volume":"10","author":"Grose","year":"2015","journal-title":"PLoS ONE"},{"key":"10.1016\/j.celrep.2017.08.018_bib19","doi-asserted-by":"crossref","first-page":"e42118","DOI":"10.1371\/journal.pone.0042118","article-title":"HSPB2 is dispensable for the cardiac hypertrophic response but reduces mitochondrial energetics following pressure overload in mice","volume":"7","author":"Ishiwata","year":"2012","journal-title":"PLoS ONE"},{"key":"10.1016\/j.celrep.2017.08.018_bib20","doi-asserted-by":"crossref","first-page":"15779","DOI":"10.1073\/pnas.0808480105","article-title":"Exploring RNA transcription and turnover in\u00a0vivo by using click chemistry","volume":"105","author":"Jao","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2017.08.018_bib21","doi-asserted-by":"crossref","first-page":"9606","DOI":"10.1073\/pnas.85.24.9606","article-title":"Replicating myoblasts express a muscle-specific phenotype","volume":"85","author":"Kaufman","year":"1988","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2017.08.018_bib22","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1212\/WNL.0b013e3181cef84a","article-title":"Mutant small heat shock protein B3 causes motor neuropathy: utility of a candidate gene approach","volume":"74","author":"Kolb","year":"2010","journal-title":"Neurology"},{"key":"10.1016\/j.celrep.2017.08.018_bib23","doi-asserted-by":"crossref","first-page":"25768","DOI":"10.1074\/jbc.M513511200","article-title":"Lamins A and C but not lamin B1 regulate nuclear mechanics","volume":"281","author":"Lammerding","year":"2006","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.celrep.2017.08.018_bib24","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1038\/nrm1172","article-title":"Nuclear speckles: a model for nuclear organelles","volume":"4","author":"Lamond","year":"2003","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib25","doi-asserted-by":"crossref","first-page":"4230","DOI":"10.1073\/pnas.0913089107","article-title":"Caspase 3\/caspase-activated DNase promote cell differentiation by inducing DNA strand breaks","volume":"107","author":"Larsen","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2017.08.018_bib26","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.dnarep.2005.10.007","article-title":"Dysfunction of lamin A triggers a DNA damage response and cellular senescence","volume":"5","author":"Lees-Miller","year":"2006","journal-title":"DNA Repair (Amst.)"},{"key":"10.1016\/j.celrep.2017.08.018_bib27","doi-asserted-by":"crossref","first-page":"e20507","DOI":"10.1371\/journal.pone.0020507","article-title":"Subcellular localization of SUN2 is regulated by lamin A and Rab5","volume":"6","author":"Liang","year":"2011","journal-title":"PLoS ONE"},{"key":"10.1016\/j.celrep.2017.08.018_bib28","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1242\/jcs.103.3.863","article-title":"Expression of wild-type and nuclear localization-deficient human lamin A in chick myogenic cells","volume":"103","author":"Lourim","year":"1992","journal-title":"J.\u00a0Cell Sci."},{"key":"10.1016\/j.celrep.2017.08.018_bib29","doi-asserted-by":"crossref","first-page":"1948","DOI":"10.1091\/mbc.e04-02-0154","article-title":"Sequestration of pRb by cyclin D3 causes intranuclear reorganization of lamin A\/C during muscle cell differentiation","volume":"16","author":"Mariappan","year":"2005","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.celrep.2017.08.018_bib30","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/S1047-8477(02)00573-7","article-title":"Increased solubility of lamins and redistribution of lamin C in X-linked Emery-Dreifuss muscular dystrophy fibroblasts","volume":"140","author":"Markiewicz","year":"2002","journal-title":"J.\u00a0Struct. Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib31","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1242\/jcs.01630","article-title":"Remodelling of the nuclear lamina and nucleoskeleton is required for skeletal muscle differentiation in\u00a0vitro","volume":"118","author":"Markiewicz","year":"2005","journal-title":"J.\u00a0Cell Sci."},{"key":"10.1016\/j.celrep.2017.08.018_bib32","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1038\/cdd.2010.183","article-title":"Prelamin A-mediated recruitment of SUN1 to the nuclear envelope directs nuclear positioning in human muscle","volume":"18","author":"Mattioli","year":"2011","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.celrep.2017.08.018_bib33","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1016\/j.cell.2015.07.047","article-title":"A liquid-to-solid phase transition of the ALS protein FUS accelerated by disease mutation","volume":"162","author":"Patel","year":"2015","journal-title":"Cell"},{"key":"10.1016\/j.celrep.2017.08.018_bib34","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1016\/j.celrep.2016.06.088","article-title":"In\u00a0vivo formation of vacuolated multi-phase compartments lacking membranes","volume":"16","author":"Schmidt","year":"2016","journal-title":"Cell Rep."},{"key":"10.1016\/j.celrep.2017.08.018_bib35","doi-asserted-by":"crossref","first-page":"3409","DOI":"10.1101\/gad.1735208","article-title":"The A- and B-type nuclear lamin networks: microdomains involved in chromatin organization and transcription","volume":"22","author":"Shimi","year":"2008","journal-title":"Genes Dev."},{"key":"10.1016\/j.celrep.2017.08.018_bib36","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1083\/jcb.200112047","article-title":"Alteration of nuclear lamin organization inhibits RNA polymerase II-dependent transcription","volume":"156","author":"Spann","year":"2002","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib37","doi-asserted-by":"crossref","first-page":"76","DOI":"10.2174\/138920312799277875","article-title":"The role of intrinsically disordered regions in the structure and functioning of small heat shock proteins","volume":"13","author":"Sudnitsyna","year":"2012","journal-title":"Curr. Protein Pept. Sci."},{"key":"10.1016\/j.celrep.2017.08.018_bib38","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1074\/jbc.275.2.1095","article-title":"Muscle develops a specific form of small heat shock protein complex composed of MKBP\/HSPB2 and HSPB3 during myogenic differentiation","volume":"275","author":"Sugiyama","year":"2000","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.celrep.2017.08.018_bib39","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1083\/jcb.147.5.913","article-title":"Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy","volume":"147","author":"Sullivan","year":"1999","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib40","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/S0014-4827(03)00092-2","article-title":"Nuclear speckle localisation of the small heat shock protein alpha B-crystallin and its inhibition by the R120G cardiomyopathy-linked mutation","volume":"287","author":"van den IJssel","year":"2003","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.celrep.2017.08.018_bib41","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1111\/j.1474-9726.2008.00382.x","article-title":"Increased mechanosensitivity and nuclear stiffness in Hutchinson-Gilford progeria cells: effects of farnesyltransferase inhibitors","volume":"7","author":"Verstraeten","year":"2008","journal-title":"Aging Cell"},{"key":"10.1016\/j.celrep.2017.08.018_bib42","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1016\/j.bbamcr.2009.05.005","article-title":"HSPB7 is a SC35 speckle resident small heat shock protein","volume":"1793","author":"Vos","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.celrep.2017.08.018_bib43","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1016\/0003-4975(94)90529-0","article-title":"Early gene changes in myocardial ischemia","volume":"58","author":"Wechsler","year":"1994","journal-title":"Ann. Thorac. Surg."},{"key":"10.1016\/j.celrep.2017.08.018_bib44","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.ceb.2015.04.003","article-title":"Nuclear bodies: the emerging biophysics of nucleoplasmic phases","volume":"34","author":"Zhu","year":"2015","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.celrep.2017.08.018_bib45","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1111\/j.1474-9726.2007.00306.x","article-title":"Cellular senescence in human myoblasts is overcome by human telomerase reverse transcriptase and cyclin-dependent kinase 4: consequences in aging muscle and therapeutic strategies for muscular dystrophies","volume":"6","author":"Zhu","year":"2007","journal-title":"Aging Cell"}],"container-title":["Cell Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124717311105?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124717311105?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T10:11:16Z","timestamp":1761905476000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2211124717311105"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,8]]},"references-count":45,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2017,8]]}},"alternative-id":["S2211124717311105"],"URL":"https:\/\/doi.org\/10.1016\/j.celrep.2017.08.018","relation":{},"ISSN":["2211-1247"],"issn-type":[{"value":"2211-1247","type":"print"}],"subject":[],"published":{"date-parts":[[2017,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function","name":"articletitle","label":"Article Title"},{"value":"Cell Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.celrep.2017.08.018","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 The Authors.","name":"copyright","label":"Copyright"}]}}