NMDI-1 increases the abundance of endogenous NMD substrates in <i>Idua</i>^W392X mice.

<p>Homozygous <i>Idua</i>^W392X mice were administered 5 mg/kg NMDI-1 for 3 days via once daily subcutaneous injections. After treatment, RNA was isolated from the brain, heart, and spleen and analyzed by RT-qPCR to determine NMD substrate steady state levels in NMDI-1 treated mice relative to untreated controls. The data shown are quantitation of the A) <i>Idua</i>, B) <i>Atf4</i>, and C) <i>Gas5</i> mRNAs normalized to 5S rRNA. Similar results were obtained when NMD substrates were normalized to 18S rRNA or <i>Rpl13a</i> (<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0060478#pone.0060478.s004" target="_blank">Figure S4</a>). The data are expressed as the fold-change in RNA levels in <i>Idua</i>^W392X mice treated with NMDI-1 relative to untreated <i>Idua</i>^W392X mice (indicated by dashed line = 1). All data are the mean +/− sd of values obtained from 3 mice per group, performed with ≥6 replicates (n = 3). <i>p</i> values above the columns compare treated to untreated mice.