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

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...

Full description

Saved in:
Bibliographic Details
Published in:Genetics (Austin) 2000-01, Vol.154 (1), p.357-362
Main Authors: Wang, Lan, Ogburn, Charles E, Ware, Carol B, Ladiges, Warren C, Youssoufian, Hagop, Martin, George M, Oshima, Junko
Format: Article
Language:English
Subjects:
4-Nitroquinoline-1-oxide - analogs & derivatives
4-Nitroquinoline-1-oxide - pharmacology
Alleles
Animals
Cell Division
DNA Helicases - genetics
Down-Regulation
Exodeoxyribonucleases
Genes, Dominant
Genetics
Humans
Mice
Mice, Transgenic
Mutation
Phenotype
Quinolones - pharmacology
RecQ Helicases
Rodents
Werner Syndrome - genetics
Werner Syndrome Helicase
wrn gene
WRN protein
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0016-6731
1943-2631
1943-2631
DOI:10.1093/genetics/154.1.357