Fig. 6.

Diminished MEF2 function and sequestration in R6/2 muscle. a Differentially expressed genes from 12 week quadriceps of R6/2 mice and 14 week quadriceps of AR113Q mice were compared to a published set of MEF2 target genes identified by gene knockdown (KD) and validated by ChIP-exo in C2C12 cells. p = 9.27e−07 for MEF2 KD vs R6/2 overlap b Differentially expressed genes from 12 week quadriceps of R6/2 mice and 14 week quadriceps from AR113Q mice were compared to a published set of gene expression changes downstream of knockdown of Mrf4, a MEF2 repressor protein, in rat tibialis anterior at 6 weeks of age. p = 6.69e−09 for Mrf4 KD vs R6/2 overlap. c Heatmap showing fold change of 280 overlapping genes in 12 week R6/2 Quad and Mrf4 KD TA. (D) R6/2 mice were examined for gene expression changes in quadriceps at 4, 8, and 12 weeks of age (n = 8–10/group for 4, 8 weeks, n = 9 WT 12 week, n = 7 R6/2 12 week). e Co-localization of p62 and MEF2A/C in quadriceps of WT and R6/2 mice at 12 weeks of age, quantified at right. Data are shown as mean ± SEM. Scale bar = 10 µm. *p < 0.05; **p < 0.01; ***p < 0.001, ****p < 0.0001 by unpaired t test