Cellular Werner Phenotypes in Mice Expressing a Putative Dominant-negative Human WRN Gene

Overview

Journal Genetics

Publisher Oxford University Press

Specialty Genetics

Date 2000 Jan 11

PMID 10628995

Citations 18

Authors

L Wang

C E Ogburn

C B Ware

W C Ladiges

H Youssoufian

G M Martin

J Oshima

Affiliations

Soon will be listed here.

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.

Citing Articles

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