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用诱导的多能干细胞建模家族癌症

Dung-Fang Lee¹, Jie Su², Huen Suk Kim²

¹Department of Developmental and Regenerative Biology and The Black Family Stem Cell Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.

|April 11, 2015

概括

诱导多能干细胞 (iPSCs) 模型李-弗劳梅尼综合征 (LFS) 骨髓瘤. 在LFS细胞中恢复H19基因表达改善了分化和降低了瘤潜力,突出显示H19.

科学领域:

干细胞生物学干细胞生物学
癌症遗传学癌症遗传学
人类疾病建模模型.

背景情况:

诱导多能干细胞 (iPSC) 技术使人体疾病的体外建模成为可能.
李-弗劳梅尼综合征 (LFS) 是一种遗传性癌症倾向综合征,通常与骨髓瘤 (OS) 相关.

研究的目的:

为了研究突变p53在骨髓瘤发育中的作用,使用来自LFS家族的患者衍生的iPSC.
探索印制基因H19在LFS相关骨髓瘤中的功能.

主要方法:

从LFS家族中确定患者衍生的iPSC.
用于体外疾病建模的iPSC衍生骨质母细胞 (OBs) 的生成.
基因表达概况和功能性基因组分析,包括整合人类印记基因网络 (IGN).

主要成果:

来自LFS iPSC的OBs表现出缺陷的骨质母细胞分化和瘤发生潜力,重述了OS特征.
在LFS OB中,基因表达特征与瘤复发率降低和患者生存率低下相关.
在LFS OB中观察到H19上调的损伤;H19的恢复促进了分化和抑制瘤性.
发现H19通过IGN组件DECORIN (DCN) 抑制了与LFS相关的OS.

结论:

来自患者的iPSC为研究LFS等人类遗传癌症综合征提供了可行的模型.
印制的H19基因通过与IGN中的DECORIN相互作用,在抑制LFS骨髓瘤发展方面发挥着至关重要的作用.

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