Immortalization of Werner syndrome and progeria fibroblasts

Exp Cell Res. 1991 Feb;192(2):373-9. doi: 10.1016/0014-4827(91)90054-x.


Human fibroblast cells from two different progeroid syndromes, Werner syndrome (WS) and progeria, were established as immortalized cell lines by transfection with plasmid DNA containing the SV40 early region. The lineage of each immortalized cell line was confirmed by VNTR analysis. Each of the immortalized cell lines maintained its original phenotype of slow growth. DNA repair ability of these cells was also studied by measuring sensitivity to killing by uv or the DNA-damaging drugs methyl methansulfonate, bleomycin, and cis-dichlorodiamine platinum. The results showed that both WS and progeria cells have normal sensitivity to these agents.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Base Sequence
  • Cell Division
  • Cell Line, Transformed / cytology*
  • Cell Transformation, Viral
  • Child, Preschool
  • DNA Damage / radiation effects
  • DNA Repair
  • Female
  • Fibroblasts / pathology*
  • Humans
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Plasmids
  • Polymorphism, Restriction Fragment Length
  • Progeria / genetics
  • Progeria / pathology*
  • Simian virus 40
  • Werner Syndrome / genetics
  • Werner Syndrome / pathology*