Kudlow Brian A, Kennedy Brian K, Monnat Raymond J
Department of Biochemistry, University of Washington, Seattle, WA 98195, USA.
Nat Rev Mol Cell Biol. 2007 May;8(5):394-404. doi: 10.1038/nrm2161.
Progeroid syndromes have been the focus of intense research in part because they might provide a window into the pathology of normal ageing. Werner syndrome and Hutchinson-Gilford progeria syndrome are two of the best characterized human progeroid diseases. Mutated genes that are associated with these syndromes have been identified, mouse models of disease have been developed, and molecular studies have implicated decreased cell proliferation and altered DNA-damage responses as common causal mechanisms in the pathogenesis of both diseases.
早老症综合征一直是深入研究的焦点，部分原因在于它们可能为正常衰老的病理学提供一个窗口。沃纳综合征和哈钦森-吉尔福德早衰综合征是两种特征最为明确的人类早老症疾病。与这些综合征相关的突变基因已被确定，疾病的小鼠模型已被开发出来，分子研究表明细胞增殖减少和DNA损伤反应改变是这两种疾病发病机制中的常见因果机制。
