肌管中MBNL1核聚集与MyoD-DM1-hiPSCs分化。(a) myod1诱导分化10天的方案示意图,参考我们之前的报告。(b)通过RT-qPCR分析dm1相关基因、DMPK和MBNL1在分化第0天、第3天和第10天mRNA表达的时间进程。基因表达量以第0天与对照414C2的相对值表示。数据代表三个独立实验的均值,采用单因素方差分析和Tukey检验进行分析(*P < 0.05, **P < 0.01, vs. 414C2对照,## P < 0.01, ## P < 0.001, vs. 409B2对照)。(c)第10天MyHC和Hoechst的代表性免疫组化图像。比例尺,100 μm。(d和e)第10天MBNL1核聚集的评估。(d) PFA或丙酮- meoh固定后MBNL1和Hoechst的代表性免疫组化图像。比例尺,10 μm。 (e) Quantitative analyses of the images of MBNL1 immunostaining after acetone-MeOH fixation using the ImageJ software. The nuclear regions were detected by Hoechst counterstaining. Data represent the means + SD of three independent experiments and were analyzed by one-way ANOVA followed by Tukey’s test (***P < 0.001). Credit:科学报告(2023)。DOI: 10.1038 / s41598 - 022 - 26614 - z