[Utilization of Werner syndrome mouse model in studying premature aging and tumor].
نویسندگان
چکیده
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 development 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.
منابع مشابه
Werner Syndrome
Werner syndrome is a premature aging disease caused by the mutation in the WRN gene. The cloning and characterization of the WRN gene and its product allows investigators to study the disease and the human aging process at molecular level. This review summarizes the recent progresses on various aspects of the WRN research including functional analysis of the protein, interactive cloning, comple...
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This perspective review focused on the Werner syndrome (WS) by addressing the issue of how a single mutation in a WRN gene encoding WRN DNA helicase induces a wide range of premature aging phenotypes accompanied by an abnormal pattern of tumors. The key event caused by WRN gene mutation is the dysfunction of telomeres. Studies on normal aging have identified a molecular circuit in which the dys...
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Werner syndrome (WS) is a human autosomal recessive genetic instability and cancer predisposition syndrome with features of premature aging. Several genetically determined mouse models of WS have been generated, however, none develops features of premature aging or an elevated risk of neoplasia unless additional genetic perturbations are introduced. In order to determine whether differences in ...
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The Werner and Bloom syndromes are caused by loss-of-function mutations in WRN and BLM, respectively, which encode the RecQ family DNA helicases WRN and BLM, respectively. Persons with Werner syndrome displays premature aging of the skin, vasculature, reproductive system, and bone, and those with Bloom syndrome display more limited features of aging, including premature menopause; both syndrome...
متن کاملLETTER TO THE EDITOR Diabetes mellitus and cancer in Werner syndrome
Onishi et al. recently reported here an association of diabetes mellitus (DM) with neoplasm type in newly identified patients with Werner syndrome (WS), an autosomal recessive cancer predisposition syndrome with features of premature aging that include a high risk of DM (Onishi et al. in Acta Diabetol 49(Suppl 1):259–260, 2012; Epstein et al. in Medicine 45:177–121, 1966). In contrast, we did n...
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ورودعنوان ژورنال:
- Yi chuan = Hereditas
دوره 31 8 شماره
صفحات -
تاریخ انتشار 2009