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. 2017 Apr 25;26(13):2541–2550. doi: 10.1093/hmg/ddx152

Figure 3.

Figure 3

12S rRNA levels are decreased in PS cells and are rescued after ectopic ERAL1 expression. (A) The 12S to 16S rRNA ratio in control and PS fibroblasts as measured by qPCR. Both patients present with significantly lower 12S/16S rRNA ratio compared to controls. Data are represented as the mean±SEM of six biological replicates. **p<0.01, ***p<0.001 as calculated by a one-way ANOVA Tukey's multiple comparisons test. (B) Western blot from PS fibroblasts infected by GFP or ERAL1 overexpressing lentivirus. Patient cells infected with ERAL1 overexpressing lentivirus stably express ERAL1 protein compared to those infected by a GFP overexpressing virus. Quantification of the levels of ERAL1 normalized to tubulin are given below the blot. (C) 12S to 16S rRNA ratio in PS fibroblasts stably expressing ERAL1 or GFP. Patient cells stably expressing ERAL1 present a significantly increased 12S/16S rRNA ratio compared to those expressing GFP, that almost reaches the levels of healthy cells (panel A). Data are represented as the mean±SEM of six biological replicates. *p<0.05, **p<0.01 as calculated by a 2-tailed student’s t-test.