Nuclear deformation characterizes Werner syndrome cells
| Autor: | Manfred Schweiger, Caroline Adelfalk, Monica Hirsch-Kauffmann, Harry Scherthan |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Biology Compound heterozygosity Cell Line medicine Humans Atypical Werner syndrome Nuclear pore Werner syndrome Cell Nucleus Centrosome Genetics Progeria integumentary system Cell Cycle nutritional and metabolic diseases Cell Biology General Medicine Lamin Type A medicine.disease Cell biology Nuclear Pore Nuclear lamina Werner Syndrome Lamin |
| Zdroj: | Cell Biology International. 29:1032-1037 |
| ISSN: | 1065-6995 |
| DOI: | 10.1016/j.cellbi.2005.10.011 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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