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Cellular Werner phenotypes in mice expressing a putative dominant-negative human WRN gene.

Genetics | Jan 17, 2000

http://www.ncbi.nlm.nih.gov/pubmed/10628995

Mutations at the Werner helicase locus (WRN) are responsible for the Werner syndrome (WS). WS patients prematurely develop an aged appearance and various age-related disorders. We have generated transgenic mice expressing human WRN with a putative dominant-negative mutation (K577M-WRN). Primary tail fibroblast cultures from K577M-WRN mice showed three characteristics of WS cells: hypersensitivity to 4-nitroquinoline-1-oxide (4NQO), reduced replicative potential, and reduced expression of the endogenous WRN protein. These data suggest that K577M-WRN mice may provide a novel mouse model for the WS.

Pubmed ID: 10628995 RIS Download

Mesh terms: 4-Nitroquinoline-1-oxide | Alleles | Animals | Cell Division | DNA Helicases | Down-Regulation | Exodeoxyribonucleases | Genes, Dominant | Humans | Mice | Mice, Transgenic | Phenotype | Quinolones | RecQ Helicases | Werner Syndrome

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Associated grants

  • Agency: NIA NIH HHS, Id: P01 AG01751
  • Agency: NIA NIH HHS, Id: R01 AG14446
  • Agency: NCI NIH HHS, Id: R24 CA78088

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