Ruzankina Yaroslava, Pinzon-Guzman Carolina, Asare Amma, Ong Tony, Pontano Laura, Cotsarelis George, Zediak Valerie P, Velez Marielena, Bhandoola Avinash, Brown Eric J
Abramson Family Cancer Research Institute, Department of Cancer Biology, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104-6160, USA.
Department of Dermatology, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104-6160, USA.
Cell Stem Cell. 2007 Jun 7;1(1):113-126. doi: 10.1016/j.stem.2007.03.002.
Developmental abnormalities, cancer, and premature aging each have been linked to defects in the DNA damage response (DDR). Mutations in the ATR checkpoint regulator cause developmental defects in mice (pregastrulation lethality) and humans (Seckel syndrome). Here we show that eliminating ATR in adult mice leads to defects in tissue homeostasis and the rapid appearance of age-related phenotypes, such as hair graying, alopecia, kyphosis, osteoporosis, thymic involution, fibrosis, and other abnormalities. Histological and genetic analyses indicate that ATR deletion causes acute cellular loss in tissues in which continuous cell proliferation is required for maintenance. Importantly, thymic involution, alopecia, and hair graying in ATR knockout mice were associated with dramatic reductions in tissue-specific stem and progenitor cells and exhaustion of tissue renewal and homeostatic capacity. In aggregate, these studies suggest that reduced regenerative capacity in adults via deletion of a developmentally essential DDR gene is sufficient to cause the premature appearance of age-related phenotypes.
发育异常、癌症和早衰均与DNA损伤反应(DDR)缺陷有关。ATR检查点调节因子的突变会导致小鼠(原肠胚形成前致死)和人类(塞克尔综合征)出现发育缺陷。在此我们表明,在成年小鼠中消除ATR会导致组织稳态缺陷,并迅速出现与年龄相关的表型,如毛发变白、脱发、脊柱后凸、骨质疏松、胸腺退化、纤维化及其他异常。组织学和遗传学分析表明,ATR缺失会导致维持组织所需持续细胞增殖的组织中出现急性细胞损失。重要的是,ATR基因敲除小鼠的胸腺退化、脱发和毛发变白与组织特异性干细胞和祖细胞的显著减少以及组织更新和稳态能力的耗尽有关。总体而言,这些研究表明,通过缺失发育必需的DDR基因来降低成体的再生能力足以导致与年龄相关表型的过早出现。