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dc.contributor.author Kim, Eun Seon -
dc.contributor.author Chung, Chang Geon -
dc.contributor.author Park, Jeong Hyang -
dc.contributor.author Ko, Byung Su -
dc.contributor.author Park, Sung Soon -
dc.contributor.author Kim, Yoon Ha -
dc.contributor.author Cha, In Jun -
dc.contributor.author Kim, Jae kwang -
dc.contributor.author Ha, Chang Man -
dc.contributor.author Kim, Hyung Jun -
dc.contributor.author Lee, Sung Bae -
dc.date.accessioned 2021-10-01T07:00:12Z -
dc.date.available 2021-10-01T07:00:12Z -
dc.date.created 2021-06-24 -
dc.date.issued 2021-06 -
dc.identifier.citation Human Molecular Genetics, v.30, no.12, pp.1084 - 1100 -
dc.identifier.issn 0964-6906 -
dc.identifier.uri http://hdl.handle.net/20.500.11750/15381 -
dc.description.abstract RNA-binding proteins (RBPs) play essential roles in diverse cellular processes through post-transcriptional regulation of RNAs. The subcellular localization of RBPs is thus under tight control, the breakdown of which is associated with aberrant cytoplasmic accumulation of nuclear RBPs such as TDP-43 and FUS, well-known pathological markers for amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). Here, we report in Drosophila model for ALS/FTD that nuclear accumulation of a cytoplasmic RBP Staufen may be a new pathological feature. We found that in Drosophila C4da neurons expressing PR36, one of the arginine-rich dipeptide repeat proteins (DPRs), Staufen accumulated in the nucleus in Importin- and RNA-dependent manner. Notably, expressing Staufen with exogenous NLS-but not with mutated endogenous NLS-potentiated PR-induced dendritic defect, suggesting that nuclear-accumulated Staufen can enhance PR toxicity. PR36 expression increased Fibrillarin staining in the nucleolus, which was enhanced by heterozygous mutation of stau (stau+/-), a gene that codes Staufen. Furthermore, knockdown of fib, which codes Fibrillarin, exacerbated retinal degeneration mediated by PR toxicity, suggesting that increased amount of Fibrillarin by stau+/- is protective. stau+/- also reduced the amount of PR-induced nuclear-accumulated Staufen and mitigated retinal degeneration and rescued viability of flies expressing PR36. Taken together, our data show that nuclear accumulation of Staufen in neurons may be an important pathological feature contributing to the pathogenesis of ALS/FTD. © The Author(s) 2021. Published by Oxford University Press. -
dc.language English -
dc.publisher Oxford University Press -
dc.title C9orf72-associated arginine-rich dipeptide repeats induce RNA-dependent nuclear accumulation of Staufen in neurons -
dc.type Article -
dc.identifier.doi 10.1093/hmg/ddab089 -
dc.identifier.wosid 000670925600002 -
dc.identifier.scopusid 2-s2.0-85108021898 -
dc.type.local Article(Overseas) -
dc.type.rims ART -
dc.description.journalClass 1 -
dc.citation.publicationname Human Molecular Genetics -
dc.contributor.nonIdAuthor Kim, Eun Seon -
dc.contributor.nonIdAuthor Chung, Chang Geon -
dc.contributor.nonIdAuthor Park, Jeong Hyang -
dc.contributor.nonIdAuthor Ko, Byung Su -
dc.contributor.nonIdAuthor Park, Sung Soon -
dc.contributor.nonIdAuthor Kim, Yoon Ha -
dc.contributor.nonIdAuthor Cha, In Jun -
dc.contributor.nonIdAuthor Kim, Jae kwang -
dc.contributor.nonIdAuthor Ha, Chang Man -
dc.contributor.nonIdAuthor Kim, Hyung Jun -
dc.identifier.citationVolume 30 -
dc.identifier.citationNumber 12 -
dc.identifier.citationStartPage 1084 -
dc.identifier.citationEndPage 1100 -
dc.identifier.citationTitle Human Molecular Genetics -
dc.description.isOpenAccess Y -
dc.subject.keywordPlus DOUBLE-STRANDED-RNAHEXANUCLEOTIDE REPEATBINDING PROTEINSRIBOSOMAL-RNANUCLEOCYTOPLASMIC TRANSPORTC9ORF72EXPANSIONLOCALIZATIONTRANSLATIONNUCLEOLUS -
dc.contributor.affiliatedAuthor Kim, Eun Seon -
dc.contributor.affiliatedAuthor Chung, Chang Geon -
dc.contributor.affiliatedAuthor Park, Jeong Hyang -
dc.contributor.affiliatedAuthor Ko, Byung Su -
dc.contributor.affiliatedAuthor Park, Sung Soon -
dc.contributor.affiliatedAuthor Kim, Yoon Ha -
dc.contributor.affiliatedAuthor Cha, In Jun -
dc.contributor.affiliatedAuthor Kim, Jae kwang -
dc.contributor.affiliatedAuthor Ha, Chang Man -
dc.contributor.affiliatedAuthor Kim, Hyung Jun -
dc.contributor.affiliatedAuthor Lee, Sung Bae -
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Department of Brain Sciences Laboratory of Neurodegenerative Diseases and Aging 1. Journal Articles

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