Figure 3. Dysregulation of protein translation does not affect cell proliferation because regulation of gene transcription remains active.

A) Western blot of whole cell lysates from patient and control fibroblasts demonstrates increased expression of MYC and decreased expression of HPRT1 in patient cells relative to controls without alterations to 4EBP1 or phosphorylated 4EBP1. B) Quantification of MYC and HPRT1 protein in control and patient fibroblasts (N = 3 for each sample) demonstrates elevation of MYC (p = 4.9×10⁻⁴) and decrease of HPRT1 protein (p = 3.5×10⁻³); bars represent mean value of each sample. C) Measurements of proliferation of patient and control fibroblasts shows no difference in cell growth (N = 3 for each measurement); error bars represent ± SEM. D) Western blots of whole cell lysates extracted from fibroblasts grown at different densities show that MYC expression is elevated in patient cells but decreases with increasing cell density. Phosphorylated 4EBP1 increases with increasing cell density E) qRT-PCR of demonstrates similar levels of MYC and HPRT1 mRNA between patient and control fibroblasts cells (N = 6); bars represent mean values ± SEM.