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Impairment of nuclear F-actin formation and its relevance to cellular phenotypes in Hutchinson-Gilford progeria syndrome
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SYSNO ASEP 0539821 Document Type J - Journal Article R&D Document Type Journal Article Subsidiary J Článek ve WOS Title Impairment of nuclear F-actin formation and its relevance to cellular phenotypes in Hutchinson-Gilford progeria syndrome Author(s) Takahashi, Y. (JP)
Hiratsuka, S. (JP)
Machida, N. (JP)
Takahashi, D. (JP)
Matsushita, J. (JP)
Hozák, Pavel (UMG-J) RID, ORCID
Misteli, T. (US)
Miyamoto, K. (JP)
Harata, M. (JP)Number of authors 9 Source Title Nucleus. - : Taylor & Francis - ISSN 1949-1034
Roč. 11, č. 1 (2020), s. 250-263Number of pages 14 s. Publication form Online - E Language eng - English Country US - United States Keywords Nuclear actin ; lamin ; nuclear organization ; Hutchinson-Gilford progeria syndrome ; progerin ; gene expression Subject RIV EB - Genetics ; Molecular Biology OECD category Cell biology R&D Projects GA19-05608S GA ČR - Czech Science Foundation (CSF) Method of publishing Open access Institutional support UMG-J - RVO:68378050 UT WOS 000571264100001 DOI 10.1080/19491034.2020.1815395 Annotation Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder caused by a mutation of lamin A, which contributes to nuclear architecture and the spatial organization of chromatin in the nucleus. The expression of a lamin A mutant, named progerin, leads to functional and structural disruption of nuclear organization. Since progerin lacks a part of the actin-binding site of lamin A, we hypothesized that nuclear actin dynamics and function are altered in HGPS cells. Nuclear F-actin is required for the organization of nuclear shape, transcriptional regulation, DNA damage repair, and activation of Wnt/beta-catenin signaling. Here we show that the expression of progerin decreases nuclear F-actin and impairs F-actin-regulated transcription. When nuclear F-actin levels are increased by overexpression of nuclear-targeted actin or by using jasplakinolide, a compound that stabilizes F-actin, the irregularity of nuclear shape and defects in gene expression can be reversed. These observations provide evidence for a novel relationship between nuclear actin and the etiology of HGPS. Workplace Institute of Molecular Genetics Contact Nikol Škňouřilová, nikol.sknourilova@img.cas.cz, Tel.: 241 063 217 Year of Publishing 2021 Electronic address https://www.tandfonline.com/doi/full/10.1080/19491034.2020.1815395
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