Brizi Valerio, Benedetti Valentina, Lavecchia Angelo Michele, Xinaris Christodoulos
IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Bergamo, Italy.
IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Bergamo, Italy.
Methods Cell Biol. 2019;153:113-132. doi: 10.1016/bs.mcb.2019.04.011. Epub 2019 May 25.
Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent, potentially lethal monogenic human disorder. There is currently no cure for ADPKD. The mechanistic complexity of the disease, the absence of animal models that can faithfully mimic the disease, as well as the lack of functional human in vitro assays for compound testing, have made drug discovery for PKD very difficult. We recently developed an engineering platform that allowed us to generate polycystic tubules using patients' own cells to test drug efficacy and discover potential new pharmacological treatments for PKD. Here we describe an engineering platform that enables the generation of custom-made polycystic tubules using patients' own cells for modeling PKD and testing drug efficacy.
常染色体显性多囊肾病(ADPKD)是最常见的、具有潜在致死性的单基因人类疾病。目前尚无治愈ADPKD的方法。该疾病机制复杂,缺乏能够忠实地模拟该疾病的动物模型,同时也缺乏用于化合物测试的功能性人体体外试验,这些都使得针对多囊肾病的药物研发变得非常困难。我们最近开发了一个工程平台,该平台使我们能够利用患者自身的细胞生成多囊小管,以测试药物疗效并发现针对多囊肾病的潜在新药物治疗方法。在此,我们描述了一个工程平台,该平台能够利用患者自身的细胞生成定制的多囊小管,用于多囊肾病建模和药物疗效测试。