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Mutations in LMNA cause a wide variety of pathologies. In this study, we analyzed the nuclear lamina of embryonic fibroblasts from LmnaH222P\/H222P mice, which develop cardiomyopathy and muscular dystrophy. Although the organization of the lamina appeared unaltered, there were changes in chromatin and B-type lamin expression. An increase in nuclear size and consequently a relative reduction in heterochromatin near the lamina allowed for a higher resolution structural analysis of lamin filaments using cryo-electron tomography. This was most apparent when visualizing lamin filaments in situ and using a nuclear extraction protocol. Averaging of individual segments of filaments in LmnaH222P\/H222P mouse fibroblasts resolved two polymers that constitute the mature filaments. Our findings provide better views of the organization of lamin filaments and the effect of a striated muscle disease-causing mutation on nuclear structure.<\/jats:p>","DOI":"10.1242\/jcs.256156","type":"journal-article","created":{"date-parts":[[2021,2,3]],"date-time":"2021-02-03T15:27:25Z","timestamp":1612366045000},"source":"Crossref","is-referenced-by-count":24,"title":["A lamin A\/C variant causing striated muscle disease provides insights into filament organization"],"prefix":"10.1242","volume":"134","author":[{"given":"Rafael","family":"Kronenberg-Tenga","sequence":"first","affiliation":[{"name":"University of Zurich 1 Department of Biochemistry , , Winterthurerstrasse 190, 8057 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meltem","family":"Tatli","sequence":"additional","affiliation":[{"name":"University of Zurich 1 Department of Biochemistry , , Winterthurerstrasse 190, 8057 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0638-9347","authenticated-orcid":false,"given":"Matthias","family":"Eibauer","sequence":"additional","affiliation":[{"name":"University of Zurich 1 Department of Biochemistry , , Winterthurerstrasse 190, 8057 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Wu","sequence":"additional","affiliation":[{"name":"Vagelos College of Physicians and Surgeons, Columbia University 2 Department of Medicine and Department of Pathology and Cell Biology , , New York, NY 10032 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji-Yeon","family":"Shin","sequence":"additional","affiliation":[{"name":"Vagelos College of Physicians and Surgeons, Columbia University 2 Department of Medicine and Department of Pathology and Cell Biology , , New York, NY 10032 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2516-3258","authenticated-orcid":false,"given":"Gis\u00e8le","family":"Bonne","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, INSERM, Centre de Recherche en Myologie, Institut de Myologie 3 , F-75651 Paris CEDEX 13 , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Howard J.","family":"Worman","sequence":"additional","affiliation":[{"name":"Vagelos College of Physicians and Surgeons, Columbia University 2 Department of Medicine and Department of Pathology and Cell Biology , , New York, NY 10032 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0994-2937","authenticated-orcid":false,"given":"Ohad","family":"Medalia","sequence":"additional","affiliation":[{"name":"University of Zurich 1 Department of Biochemistry , , Winterthurerstrasse 190, 8057 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2021,3,22]]},"reference":[{"key":"2024061307434480100_JCS256156C1","doi-asserted-by":"publisher","first-page":"3757","DOI":"10.1038\/s41467-019-11684-x","article-title":"Structural basis for lamin assembly at the molecular level","volume":"10","author":"Ahn","year":"2019","journal-title":"Nat. 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