{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T19:38:12Z","timestamp":1785181092984,"version":"3.55.0"},"reference-count":35,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2014,10,16]],"date-time":"2014-10-16T00:00:00Z","timestamp":1413417600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes to Cells"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:p>Mutation of the lamin A gene (<jats:italic><jats:styled-content style=\"fixed-case\">LMNA<\/jats:styled-content><\/jats:italic>) causes a diverse range of diseases referred to as laminopathies. Because most laminopathies have a dominant inheritance pattern and progress gradually, cultured cells stably expressing mutant lamin A at the same level as endogenous wild\u2010type cells are required for chronological analysis. In this study, we showed that an expression system involving a lentiviral vector that carries the human metallothionein gene basal promoter ensures stable and basal\u2010level expression of proteins and is thus suitable for investigating the properties of lamin A mutants. The small ubiquitin\u2010related modifier (<jats:styled-content style=\"fixed-case\">SUMO<\/jats:styled-content>) modification (<jats:styled-content style=\"fixed-case\">SUMO<\/jats:styled-content>ylation)\u2010defective E203G mutant that is associated with familial dilated cardiomyopathy exhibited abnormal subnuclear distribution and inhibited normal localization of <jats:styled-content style=\"fixed-case\">WT<\/jats:styled-content> lamin A in a dominant\u2010negative manner. Low\u2010level and long\u2010term expression of the E203G mutant resulted in multinucleated giant cells, aberrant lipid droplet accumulation in the cytoplasm and premature senescence. Expression of another <jats:styled-content style=\"fixed-case\">SUMO<\/jats:styled-content>ylation\u2010defective mutant (K201R) did not induce any phenotypes observed in cells expressing E203G. These results indicate that the E203G mutant may inhibit the normal functions of wild\u2010type lamin A in a dominant\u2010negative manner, but a defect in <jats:styled-content style=\"fixed-case\">SUMO<\/jats:styled-content>ylation itself may not be involved in disease pathogenesis.<\/jats:p>","DOI":"10.1111\/gtc.12189","type":"journal-article","created":{"date-parts":[[2014,10,16]],"date-time":"2014-10-16T08:35:47Z","timestamp":1413448547000},"page":"901-918","source":"Crossref","is-referenced-by-count":7,"title":["Long\u2010term expression of the lamin <scp>A<\/scp> mutant associated with dilated cardiomyopathy induces senescence"],"prefix":"10.1111","volume":"19","author":[{"given":"Takanobu","family":"Moriuchi","sequence":"first","affiliation":[{"name":"Graduate School of Life Science University of Hyogo Koto Kamigori, Hyogo 678\u20101297 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