Impairment of nuclear F-actin formation and its relevance to cellular phenotypes in Hutchinson-Gilford progeria syndrome

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F68378050%3A_____%2F20%3A00539821" target="_blank" >RIV/68378050:_____/20:00539821 - isvavai.cz</a>

Result on the web

<a href="https://www.tandfonline.com/doi/full/10.1080/19491034.2020.1815395" target="_blank" >https://www.tandfonline.com/doi/full/10.1080/19491034.2020.1815395</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1080/19491034.2020.1815395" target="_blank" >10.1080/19491034.2020.1815395</a>

Result language

angličtina

Original language name

Impairment of nuclear F-actin formation and its relevance to cellular phenotypes in Hutchinson-Gilford progeria syndrome

Original language description

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.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

10601 - Cell biology

Project

<a href="/en/project/GA19-05608S" target="_blank" >GA19-05608S: Functions of lamin A - phosphoinositides complexes in the cell nucleus.</a><br>

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2020

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Nucleus

ISSN

1949-1034

e-ISSN

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Volume of the periodical

11

Issue of the periodical within the volume

1

Country of publishing house

US - UNITED STATES

Number of pages

14

Pages from-to

250-263

UT code for WoS article

000571264100001

EID of the result in the Scopus database

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