The transcriptional response to distinct growth factors is impaired in Werner syndrome cells

Exp Gerontol. 2008 Sep;43(9):820-6. doi: 10.1016/j.exger.2008.06.007. Epub 2008 Jun 25.

Abstract

The Werner syndrome protein (WRN) is mutated in Werner syndrome (WS) and plays a role in telomere maintenance, DNA repair and transcription. WS represents a premature aging syndrome with severe growth retardation. Here we show that WRN is critically required to mediate the stimulatory effect of vascular endothelial growth factor (VEGF), basic fibroblast growth factor (FGF-b) and epidermal growth factor (EGF) on the activity of RNA polymerase I (Pol I). Recombinant WRN specifically reconstitutes RNA polymerase I transcription in extracts from Werner syndrome fibroblasts in vitro. In addition, we identified a critical role for WRN during promoter clearance of Pol I transcription, but not in elongation. Notably, WRN was isolated in a complex with Pol I and was crosslinked to the unmethylated, active proportion of rDNA genes in quiescent cells suggesting a so far unknown role for WRN in epigenetic regulation. This together with alterations in Pol I transcription provide a novel mechanism possibly underlying at least in part the severe growth retardation and premature aging in Werner syndrome patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cells, Cultured
  • DNA Methylation
  • DNA, Ribosomal / metabolism
  • DNA-Binding Proteins / metabolism
  • Exodeoxyribonucleases / genetics
  • Exodeoxyribonucleases / metabolism
  • Exodeoxyribonucleases / pharmacology
  • Fibroblasts / drug effects
  • Fibroblasts / pathology
  • Humans
  • Intercellular Signaling Peptides and Proteins / pharmacology*
  • Mutation
  • Promoter Regions, Genetic
  • RNA Polymerase I / genetics
  • RecQ Helicases / genetics
  • RecQ Helicases / metabolism
  • RecQ Helicases / pharmacology
  • Recombinant Proteins / pharmacology
  • Transcription, Genetic / drug effects*
  • Werner Syndrome / genetics*
  • Werner Syndrome / metabolism
  • Werner Syndrome / pathology
  • Werner Syndrome Helicase

Substances

  • DNA, Ribosomal
  • DNA-Binding Proteins
  • Intercellular Signaling Peptides and Proteins
  • Recombinant Proteins
  • RNA Polymerase I
  • Exodeoxyribonucleases
  • RecQ Helicases
  • WRN protein, human
  • Werner Syndrome Helicase