Extended Data Fig. 9: AGR-H19 and Nifenazone restore skeletal muscle and heart function.
From: The lncRNA H19 alleviates muscular dystrophy by stabilizing dystrophin
a, Immunohistological detection of Biotin-labeled mimics in quadriceps, heart, kidney, liver and lung tissues in the presence of indicated mimics. Scale bars, 100 μm. Data represent independent replicates using 5 animals per group. b, Body weight measurement of male Dmd WT treated with scramble (Scr) or Dmd C3333Y mice with indicated treatments. Mean values±SD, n = 5 mice per group, two-way ANOVA. c, d, Serum IGF1 (c) and IGF2 (d) level concentration of Dmd WT or Dmd C3333Y mice with the indicated treatments. Mean values±SD, n = 5 mice per group, one-way ANOVA. e, RT-qPCR detection of Dmd in Dmd WT or Dmd C3333Y QUA (quadriceps) with indicated treatments. Mean values±SD, n = 6 mice in each group, one-way ANOVA. f-i, RT-qPCR detection of indicated genes in Dmd C3333Y QUA with indicated treatments. Mean values±SD, n = 5 (f, g, h) or 6 (i) mice in each group, one-way ANOVA. j, Serum CK concentration in Dmd WT treated with AGR-Scr or Dmd C3333Y mice with indicated treatments. Mean values±SD, n = 5 mice per group, one-way ANOVA. k, Percentage of central nucleic of indicated muscle fiber of Dmd WT treated with AGR-Scr or Dmd C3333Y mice with indicated treatments. Mean values±SD, n = 5 mice per group, two-way ANOVA. l, Body weight measurement of male Dmd WT mice treated with vehicle or Dmd C3333Y mice with indicated treatments from 4 weeks old to age of 16 weeks. Mean values±SD, n = 5 mice per group, two-way ANOVA. m, Serum CK concentration in Dmd WT mice treated with vehicle or Dmd C3333Y mice with indicated treatments. Mean values±SD, n = 5 mice per group, one-way ANOVA. No significance [n.s.], p > 0.05, *, p < 0.05, **, p < 0.01, ***, p < 0.001, and ****, p < 0.0001. Statistical source data are provided as Source Data Extended Data Fig. 9.
