Werner综合征小鼠模型在早衰与肿瘤研究中的应用

贾舒婷，杨世华，罗瑛

doi:10.3724/SP.J.1005.2009.00785

遗传 >

2009 , Vol. 31 >Issue 8: 785 - 790

DOI: https://doi.org/10.3724/SP.J.1005.2009.00785

综述

Werner综合征小鼠模型在早衰与肿瘤研究中的应用

展开

昆明理工大学生命科学与技术学院, 衰老与肿瘤分子遗传学实验室, 昆明 650224

收稿日期: 2008-09-19

修回日期: 2009-04-16

网络出版日期: 2009-08-10

收起

Utilization of Werner syndrome mouse model in studying premature aging and tumor

Expand

Laboratory of Molecular Genetics of Aging and Tumor, Faculty of Life Science and Technology, Kunming University of Science and Technology, Kunming 650224, China

Received date: 2008-09-19

Revised date: 2009-04-16

Online published: 2009-08-10

Fold

摘要

Werner综合征(Werner syndrome, WS)是一种罕见的人类常染色体隐性遗传疾病, 一直以来该病作为研究人类早老综合征的典型病例而受到关注。Werner蛋白(WRN)是Werner综合征中突变的核蛋白, 最近的生化及遗传学研究证明WRN在DNA复制、DNA损伤修复以及端粒的维持方面起着重要的作用。文章综述了Werner综合征的分子遗传学机理及端粒和WRN在Werner综合征发病中的重要作用。通过双敲除Wrn与端粒酶基因建立的小鼠模型忠实地再现了人类Werner综合征, 这种Werner综合征小鼠模型因其同时具有早衰与肿瘤表型而在研究人类肿瘤及衰老的相关性中起到的独特作用。

关键词： 早衰; 端粒; 肿瘤; 基因敲除小鼠模型; Werner综合征

本文引用格式

贾舒婷，杨世华，罗瑛 . Werner综合征小鼠模型在早衰与肿瘤研究中的应用[J]. 遗传, 2009 , 31(8) : 785 -790 . DOI: 10.3724/SP.J.1005.2009.00785

Abstract

Werner syndrome (WS) is a rare autosomal recessive genetic disease in human. It is considered as a good model disease in studying human premature syndrome. Werner protein (WRN) is a nuclear protein mutated in WS. Recent biochemical and genetic studies indicated that WRN plays important roles in DNA replication, DNA repair, and telomere maintenance. Here, we reviewed the molecular genetics of WS and the importance of telomere and WRN in the develop-ment of WS. Knocking out both telomerase and Wrn genes in mouse faithfully manifests human WS. The mouse model provides a unique genetic platform to explore the crosstalk of premature aging and tumor.

Options

文章导航

〈

〉

www.chinagene.cn
备案号：京ICP备09063187号-4
总访问:,今日访问:,当前在线:

模态框（Modal）标题

摘要

本文引用格式

Abstract