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

First Author  Wang L Year  2000
Journal  Genetics Volume  154
Issue  1 Pages  357-62
PubMed ID  10628995 Mgi Jnum  J:95127
Mgi Id  MGI:3525113 Doi  10.1093/genetics/154.1.357
Citation  Wang L, et al. (2000) Cellular Werner phenotypes in mice expressing a putative dominant-negative human WRN gene. Genetics 154(1):357-62
abstractText  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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