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. 2000 Jan;154(1):357–362. doi: 10.1093/genetics/154.1.357

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

L Wang 1, C E Ogburn 1, C B Ware 1, W C Ladiges 1, H Youssoufian 1, G M Martin 1, J Oshima 1
PMCID: PMC1460888  PMID: 10628995

Abstract

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.

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Selected References

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