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Lamins, along with their interactors, act as determinants for chromatin organization throughout the nucleus. The major dominant missense mutations responsible for autosomal dominant forms of muscular dystrophies reside in the Ig fold domain of lamin A. However, how lamin A contributes to the distribution of heterochromatin and balances euchromatin, and how it relocates epigenetic marks to shape chromatin states, remains poorly defined, making it difficult to draw conclusions about the prognosis of lamin A-mediated muscular dystrophies. In the first part of this report, we identified the in vitro organization of full-length lamin A proteins due to two well-documented Ig LMNA mutations, R453W and W514R. We further demonstrated that both lamin A\/C mutant cells predominantly expressed nucleoplasmic aggregates. Labeling specific markers of epigenetics allowed correlation of lamin A mutations with epigenetic mechanisms. In addition to manipulating epigenetic mechanisms, our proteomic studies traced diverse expressions of transcription regulators, RNA synthesis and processing proteins, protein translation components, and posttranslational modifications. These data suggest severe perturbations in targeting other proteins to the nucleus.<\/jats:p>","DOI":"10.1042\/bcj20240474","type":"journal-article","created":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T12:30:31Z","timestamp":1730982631000},"page":"1803-1827","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Investigating the differential structural organization and gene expression regulatory networks of lamin A Ig fold domain mutants of muscular dystrophy"],"prefix":"10.1042","volume":"481","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8103-5771","authenticated-orcid":false,"given":"Subarna","family":"Dutta","sequence":"first","affiliation":[{"name":"1Department of Biochemistry, University of Calcutta, 35, Ballygunge Circular Road, Kolkata 700019, West Bengal, India"},{"name":"2Theomics International Private Limited 28, Income Tax Layout, Sadananda Nagar, NGEF Layout, Bengaluru 560038, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikas","family":"Kumar","sequence":"additional","affiliation":[{"name":"3UMass Chan Medical School, Mass Spectrometry Facility, 222 Maple Avenue, Shrewsbury, MA 01545, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnab","family":"Barua","sequence":"additional","affiliation":[{"name":"4Tata Institute of Fundamental Research, Hyderabad 500046, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madavan","family":"Vasudevan","sequence":"additional","affiliation":[{"name":"2Theomics International Private Limited 28, Income Tax Layout, Sadananda Nagar, NGEF Layout, Bengaluru 560038, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2024,11,28]]},"reference":[{"key":"2024112907313068200_BCJ-2024-0474C1","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1126\/science.1439805","article-title":"The nuclear membrane","volume":"258","year":"1992","journal-title":"Science"},{"key":"2024112907313068200_BCJ-2024-0474C2","doi-asserted-by":"publisher","first-page":"142","DOI":"10.4161\/nucl.24089","article-title":"Disruption of lamin B1 and lamin B2 processing and localization by farnesyltransferase inhibitors","volume":"4","year":"2013","journal-title":"Nucleus"},{"key":"2024112907313068200_BCJ-2024-0474C3","doi-asserted-by":"publisher","first-page":"1223","DOI":"10.1083\/jcb.200303113","article-title":"A carboxyl-terminal interaction of lamin B1 is dependent on the CAAX endoprotease Rce1 and carboxymethylation","volume":"162","year":"2003","journal-title":"J. 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