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Nuclear deformation characterizes Werner syndrome cells.

Abstract：

:Mutations in the lamin A gene have been shown, among other defects, to give rise to Hutchinson-Gilford progeria syndrome (HGPS) and to atypical Werner syndrome (WS), both of which are progeroid disorders. Here, we have investigated well-characterized WS patient cell strains that are compound heterozygous for mutations in the WRN gene. As in HGPS and in atypical WS, we found nuclear deformations to be characteristic of all cell strains studied. In WS cells centrosome number, assembly of the nuclear lamina and nuclear pore distribution occurred normally. Furthermore, nuclear deformations were not associated with a defect in lamin A expression. We propose that nuclear deformation is a universal characteristic of progeroid cells and may result from slow cell cycle progression.

journal_name

Cell Biol Int

journal_title

Cell biology international

authors

Adelfalk C,Scherthan H,Hirsch-Kauffmann M,Schweiger M

doi

10.1016/j.cellbi.2005.10.011

keywords:

subject

Has Abstract

pub_date

2005-12-01 00:00:00

pages

1032-7

issue

12

eissn

1065-6995

issn

1095-8355

pii

S1065-6995(05)00243-X

journal_volume

29

pub_type

杂志文章

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