Fig. 7.

CA-MEF2 rescues AR113Q muscle atrophy and target gene expression. a–d AR113Q mice were transfected with CA-MEF2 in one leg and DBD-MEF2 in the contralateral leg. Each plasmid was co-transfected with GFP for fiber identification. After 24 h, mice were injected with tamoxifen every other day for 7 days.a Tibialis anterior was harvested and imaged for GFP (green), Wheat Germ Agglutinin (WGA) (red) and DAPI (blue). Scale bar = 50 μM. b Tibialis anterior was stained using Hematoxylin and Eosin (H&E). Scale bar = 50 μM. c, d Quantification of cross sectional area of transfected fibers (n = 3mice/group, 3 images/mouse, n > 50 fibers/mouse) is shown as a histogram (c) and mean ± SEM (d), ****p < 0.0001 by unpaired t test. e, f AR113Q muscle expressing DBD-MEF2 and CA-MEF2 was examined for expression levels of e MEF2 targets found to be altered in SBMA patients Ttn, MyoZ1, Pfkm, Clcn1 and fgenes that regulate metabolism including Gapdh, Hk2, and Nur77 (n = 6 mice/group). Data are mean ± SEM. *p < 0.05; ***p < 0.001 by unpaired t test