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Here, we provide new evidence showing that increased lamin B1 levels contribute to the pathophysiology of Huntington\u2019s disease (HD), a CAG repeat\u2010associated neurodegenerative disorder. Through fluorescence\u2010activated nuclear suspension imaging, we show that nucleus from striatal medium\u2010sized spiny and CA1 hippocampal neurons display increased lamin B1 levels, in correlation with altered nuclear morphology and nucleocytoplasmic transport disruption. Moreover, ChIP\u2010sequencing analysis shows an alteration of lamin\u2010associated chromatin domains in hippocampal nuclei, accompanied by changes in chromatin accessibility and transcriptional dysregulation. Supporting lamin B1 alterations as a causal role in mutant huntingtin\u2010mediated neurodegeneration, pharmacological normalization of lamin B1 levels in the hippocampus of the R6\/1 mouse model of HD by betulinic acid administration restored nuclear homeostasis and prevented motor and cognitive dysfunction. Collectively, our work points increased lamin B1 levels as a new pathogenic mechanism in HD and provides a novel target for its intervention.<\/jats:p>","DOI":"10.15252\/emmm.202012105","type":"journal-article","created":{"date-parts":[[2020,12,28]],"date-time":"2020-12-28T09:13:32Z","timestamp":1609146812000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["Neuron type\u2010specific increase in lamin B1 contributes to nuclear dysfunction in Huntington\u2019s disease"],"prefix":"10.1038","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3540-645X","authenticated-orcid":false,"given":"Rafael","family":"Alcal\u00e1\u2010Vida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3505-1003","authenticated-orcid":false,"given":"Marta","family":"Garcia\u2010Forn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5812-6683","authenticated-orcid":false,"given":"Carla","family":"Castany\u2010Pladevall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2572-1560","authenticated-orcid":false,"given":"Jordi","family":"Creus\u2010Muncunill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4578-6126","authenticated-orcid":false,"given":"Yoko","family":"Ito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6261-7370","authenticated-orcid":false,"given":"Enrique","family":"Blanco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0368-5538","authenticated-orcid":false,"given":"Arantxa","family":"Golbano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3346-9513","authenticated-orcid":false,"given":"Kilian","family":"Cresp\u00ed\u2010V\u00e1zquez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5192-3727","authenticated-orcid":false,"given":"Aled","family":"Parry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4202-8285","authenticated-orcid":false,"given":"Guy","family":"Slater","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1046-0601","authenticated-orcid":false,"given":"Shamith","family":"Samarajiwa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9519-3877","authenticated-orcid":false,"given":"Sandra","family":"Peir\u00f3","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3488-6228","authenticated-orcid":false,"given":"Luciano","family":"Di Croce","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7764-577X","authenticated-orcid":false,"given":"Masashi","family":"Narita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9165-6539","authenticated-orcid":false,"given":"Esther","family":"P\u00e9rez\u2010Navarro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,12,28]]},"reference":[{"key":"BFEMMM202012105_CR1","doi-asserted-by":"crossref","first-page":"6250","DOI":"10.1007\/s12035-017-0828-4","volume":"55","author":"E Alvarez\u2010Periel","year":"2018","unstructured":"Alvarez\u2010Periel E, Puigdell\u00edvol M, Brito V, Plattner F, Bibb JA, Alberch J, Gin\u00e9s S (2018) Cdk5 contributes to huntington\u2019s disease learning and memory deficits via modulation of brain region\u2010specific substrates. 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