诱导多能干细胞
Wnt信号通路
类有机物
大肠腺瘤性息肉病
干细胞
生物
癌症研究
胚胎干细胞
家族性腺瘤性息肉病
结直肠癌
癌症干细胞
细胞生物学
分子生物学
癌症
信号转导
遗传学
基因
作者
Miguel Crespo,Eduardo Vilar,Su‐Yi Tsai,Kyle Chang,Sadaf Amin,Tara Srinivasan,Tuo Zhang,Nina H. Pipalia,Huanhuan Joyce Chen,Mavee Witherspoon,Miriam Gordillo,Jenny Xiang,Frederick R. Maxfield,Steven M. Lipkin,Todd Evans,Shuibing Chen
出处
期刊:Nature Medicine
[Nature Portfolio]
日期:2017-06-19
卷期号:23 (7): 878-884
被引量:386
摘要
With the goal of modeling human disease of the large intestine, we sought to develop an effective protocol for deriving colonic organoids (COs) from differentiated human embryonic stem cells (hESCs) or induced pluripotent stem cells (iPSCs). Extensive gene and immunohistochemical profiling confirmed that the derived COs represent colon rather than small intestine, containing stem cells, transit-amplifying cells, and the expected spectrum of differentiated cells, including goblet and endocrine cells. We applied this strategy to iPSCs derived from patients with familial adenomatous polyposis (FAP-iPSCs) harboring germline mutations in the WNT-signaling-pathway-regulator gene encoding APC, and we generated COs that exhibit enhanced WNT activity and increased epithelial cell proliferation, which we used as a platform for drug testing. Two potential compounds, XAV939 and rapamycin, decreased proliferation in FAP-COs, but also affected cell proliferation in wild-type COs, which thus limits their therapeutic application. By contrast, we found that geneticin, a ribosome-binding antibiotic with translational 'read-through' activity, efficiently targeted abnormal WNT activity and restored normal proliferation specifically in APC-mutant FAP-COs. These studies provide an efficient strategy for deriving human COs, which can be used in disease modeling and drug discovery for colorectal disease.
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