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从诱导多能干细胞 (iPSC) 来进行神经分化和脊髓器官生成,用于ALS建模和炎症查

Ruiyun Guo^1,2,3, Yimeng Chen^1,2,3, Jinyu Zhang^1,2,3

¹Hebei Medical University-University of Galway Stem Cell Research Center, Hebei Medical University, Shijiazhuang, 050017, Hebei Province, China.

Molecular neurobiology

|December 21, 2023

概括

一个C9orf72基因突变,ALS/FTD的常见原因,使用人类诱导的多能干细胞 (iPSCs) 建模. 这些iPSC产生的脊髓器官 (SCOs) 揭示神经炎症作为一个关键的病理特征.

科学领域:

神经科学是一个神经科学.
遗传学是一个遗传学.
干细胞生物学干细胞生物学

背景情况:

C9orf72基因突变是肌缩侧面硬化症 (ALS) 和前性痴呆症 (FTD) 的主要遗传原因.
诱导多能干细胞 (iPSCs) 为疾病建模提供了一个有前途的平台.
了解C9orf72相关的神经退行症背后的细胞和分子机制至关重要.

研究的目的:

建立和描述人类iPSC衍生的神经元模型,包括携带C9orf72突变的脊髓器官 (SCOs).
研究与C9orf72缺乏相关的病态表型,重点关注神经炎症.
利用这些模型进行查和了解疾病机制.

主要方法:

从纤维细胞捐赠者生成野生类型 (WT) 和C9orf72-Knockdown人类iPSC线条.
使用RT-PCR,免疫光和流细胞计,对iPSC多能性和差异化潜力的表征.
将iPSC分化为星体细胞,运动神经元和3D脊髓有机体 (SCO).
来自iPSC衍生物细胞的RNA-seq数据和qRT-PCR用于炎症因子表达的生物信息分析.

主要成果:

WT和C9orf72-knockdown iPSCs表现出类似的分化能力,分为生殖层,星细胞,运动神经元和SCOs.
C9orf72-knockdown SCOs重述了脊髓发育特征,并包含了各种神经元亚型.
生物信息分析和qRT-PCR证实了来自C9orf72-突变iPSCs的二维iPSC培养物和三维SCOs中的促炎因素的上调.
这些模型显示C9orf72表达减少和ALS的关键病理标志,特别是神经炎症.

结论:

人类iPSC衍生的2D神经元培养和3DSCO是研究C9orf72相关的ALS/FTD的有效模型.
神经炎症是C9orf72相关疾病模型中重要的病理特征.
这些模型为进一步研究疾病机制和潜在的治疗查提供了宝贵的平台.

关键词:

肌缩侧面硬化症 (ALS) 是一种疾病.C9orf72;脊髓有机体 (SCOs) 是一种有机体.诱导多能干细胞 (iPSCs) 是一种干细胞.

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