Rudolph K L, Chang S, Millard M, Schreiber-Agus N, DePinho R A
Department of Adult Oncology, Medicine and Genetics, Dana-Farber Cancer Institute, 44 Binney Street (M413), and Harvard Medical School, Boston, MA 02115, USA.
Science. 2000 Feb 18;287(5456):1253-8. doi: 10.1126/science.287.5456.1253.
Accelerated telomere loss has been proposed to be a factor leading to end-stage organ failure in chronic diseases of high cellular turnover such as liver cirrhosis. To test this hypothesis directly, telomerase-deficient mice, null for the essential telomerase RNA (mTR) gene, were subjected to genetic, surgical, and chemical ablation of the liver. Telomere dysfunction was associated with defects in liver regeneration and accelerated the development of liver cirrhosis in response to chronic liver injury. Adenoviral delivery of mTR into the livers of mTR(-/-) mice with short dysfunctional telomeres restored telomerase activity and telomere function, alleviated cirrhotic pathology, and improved liver function. These studies indicate that telomere dysfunction contributes to chronic diseases of continual cellular loss-replacement and encourage the evaluation of "telomerase therapy" for such diseases.
端粒加速损耗被认为是导致高细胞更新率的慢性疾病(如肝硬化)终末期器官衰竭的一个因素。为了直接验证这一假设,对端粒酶RNA(mTR)基因缺失的端粒酶缺陷小鼠进行了肝脏的基因、手术和化学切除。端粒功能障碍与肝脏再生缺陷相关,并加速了慢性肝损伤后肝硬化的发展。将mTR通过腺病毒载体导入端粒功能失调且较短的mTR(-/-)小鼠肝脏中,可恢复端粒酶活性和端粒功能,减轻肝硬化病理变化,并改善肝功能。这些研究表明,端粒功能障碍促成了持续细胞损耗-替代的慢性疾病,并鼓励对这类疾病进行“端粒酶疗法”的评估。