Nuclear deformation characterizes Werner syndrome cells.
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.
Adelfalk
Caroline
C
Max-Planck-Institute for Molecular Genetics, Ihnestrasse 73, 14195 Berlin, Germany. adelfalk@web.de
Scherthan
Harry
H
Hirsch-Kauffmann
Monica
M
Schweiger
Manfred
M
D002453:Cell Cycle; D002460:Cell Line; D002467:Cell Nucleus / Q000473:pathology*; D018385:Centrosome / Q000378:metabolism; D006801:Humans; D034904:Lamin Type A / Q000378:metabolism*; D022022:Nuclear Pore / Q000378:metabolism*; D014898:Werner Syndrome / Q000473:pathology*
D034904:Lamin Type A