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The cellular phenomena associated with laminopathy invariably show disruption of nucleoskeleton of lamina due to deregulated expression, localization, function, and interaction of mutant lamin proteins. Impaired spatial and temporal tethering of lamin proteins to the lamina or nucleoplasmic aggregation of lamins are the primary molecular events that can trigger nuclear proteotoxicity by modulating differential protein\u2013protein interactions, sequestering quality control proteins, and initiating a cascade of abnormal post\u2010translational modifications. Clearly, laminopathic cells exhibit moderate to high nuclear proteotoxicity, raising the question of whether an imbalance in nuclear proteostasis is involved in laminopathic diseases, particularly in diseases of early aging such as HGPS and laminopathy\u2010associated premature aging. Here, we review nuclear proteostasis and its deregulation in the context of lamin proteins and laminopathies.<\/jats:p>","DOI":"10.1096\/fj.202300878r","type":"journal-article","created":{"date-parts":[[2023,7,25]],"date-time":"2023-07-25T09:51:17Z","timestamp":1690278677000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Nuclear proteostasis imbalance in laminopathy\u2010associated premature aging diseases"],"prefix":"10.1096","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5700-7789","authenticated-orcid":false,"given":"Shruti","family":"Pande","sequence":"first","affiliation":[{"name":"Department of Medical Genetics Kasturba Medical College, Manipal, Manipal Academy of Higher Education  Manipal India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9196-0685","authenticated-orcid":false,"given":"Debasish 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