Extended Data Fig. 7: H19 and Nifenazone extend the half-life of DMD protein.
From: The lncRNA H19 alleviates muscular dystrophy by stabilizing dystrophin
a, Representative images of IF staining using indicated antibody (top) and statistical analysis of DMD staining intensities (bottom) of hiPS-CMs derived from healthy donor or BMD patients, upon transfection of Scramble (Scr) mimic, H19 mimic or treatment Nifenazone (10 µM). Scale bars, 50 µm. Mean values±SD, n = 5 independent experiments, two-way ANOVA. b, Representative images of IF staining using indicated antibody (top) and statistical analysis of Ub-DMD (Lys3584) staining intensities (bottom) of hiPS-CMs derived from healthy donor or BMD patients, upon transfection of Scramble (Scr) mimic, H19 mimic or treatment Nifenazone (10 µM). Scale bars, 50 µm. Mean values±SD, n = 5 independent experiments, two-way ANOVA. c, IP using DMD antibody followed by IB detection using indicated antibodies in hiPS-SkMCs from healthy donor or BMD patients with the indicated treatments. d–g, RT-QCPR detection of indicated genes of hiPS-SkMCs derived from healthy donor or BMD patients, upon transfection of Scramble (Scr) mimic, H19 mimic or treatment Nifenazone (10 µM). Mean values±SD, n = 5 independent experiments, two-way ANOVA. No significance [n.s.], p > 0.05, *, p < 0.05, **, p < 0.01, ***, p < 0.001, ****, p < 0.0001. Immunoblots are representative of two independent experiments. Statistical source data and unprocessed immunoblots are provided as Source Data Extended Data Fig. 7.
