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The mechanisms of toxicity that result in the complex HD pathology remain only partially understood. Here, we show that nuclear polyglutamine aggregates induce nuclear envelope (NE) blebbing and ruptures that are often repaired incompletely. These ruptures coincide with disruptions of the nuclear lamina and lead to lamina scar formation. Expansion microscopy enabled resolving the ultrastructure of nuclear aggregates and revealed polyglutamine fibrils sticking into the cytosol at rupture sites, suggesting a mechanism for incomplete repair. Furthermore, we found that NE repair factors often accumulated near nuclear aggregates, consistent with stalled repair. These findings implicate nuclear polyQ aggregate-induced loss of NE integrity as a potential contributing factor to Huntington\u2019s disease and other polyglutamine diseases.<\/jats:p>","DOI":"10.1083\/jcb.202307142","type":"journal-article","created":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T02:21:18Z","timestamp":1722392478000},"update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":17,"title":["Nuclear poly-glutamine aggregates rupture the nuclear envelope and hinder its repair"],"prefix":"10.1083","volume":"223","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-6076-2929","authenticated-orcid":true,"given":"Giel","family":"Korsten","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-6901-0733","authenticated-orcid":true,"given":"Miriam","family":"Osinga","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7342-3034","authenticated-orcid":true,"given":"Robin A.","family":"Pelle","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-5147-3664","authenticated-orcid":true,"given":"Albert K.","family":"Serweta","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-2386-2999","authenticated-orcid":true,"given":"Baukje","family":"Hoogenberg","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8966-8466","authenticated-orcid":true,"given":"Harm H.","family":"Kampinga","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/03cv38k47","id-type":"ROR","asserted-by":"publisher"}],"name":"University of Groningen 2 Department of Cell Biology, University Medical Center Groningen, , Groningen, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9418-6739","authenticated-orcid":true,"given":"Lukas C.","family":"Kapitein","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"Utrecht University 1 Cell Biology, Neurobiology and Biophysics, Department of Biology, Faculty of Science, , Utrecht, Netherlands"},{"id":[{"id":"https:\/\/ror.org\/04pp8hn57","id-type":"ROR","asserted-by":"publisher"}],"name":"UMC Utrecht University 3 Centre for Living Technologies, Alliance TU\/e, WUR, UU, , Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2024,8,16]]},"reference":[{"key":"2024081613435613700_bib1","doi-asserted-by":"publisher","first-page":"691","DOI":"10.1007\/s00401-011-0911-2","article-title":"p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND\/ALS","volume":"122","author":"Al-Sarraj","year":"2011","journal-title":"Acta Neuropathol."},{"key":"2024081613435613700_bib2","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.202012105","article-title":"Neuron type-specific increase in lamin B1 contributes to nuclear dysfunction in Huntington\u2019s disease","volume":"13","author":"Alcal\u00e1-Vida","year":"2021","journal-title":"EMBO Mol. 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