Fig. 5

WDR4 patient fibroblasts are not deficient in protein synthesis.a Nascent protein synthesis is increased in WDR4 patient fibroblasts under cycling and resting conditions. n = 4?5. p = 0.0038 (**) and 0.0002 (***). Welch?s t test. b Overall protein content is not changed in WDR4 patient fibroblasts. n = 5?6. Mann?Whitney test. c WDR4 patient cells do not have more premature abortions during protein synthesis. Bar graph shows the distribution of peptides detected by mass spectrometry along the whole length of the protein. n = 2. d Assessment of translation fidelity in control and WDR4 patient fibroblasts using a luciferase based assay. Cells were transfected with firefly luciferase reporter constructs including the indicated mutations in the active center of the luciferase. Elevated luciferase activity indicates erroneous translation and reduced translational fidelity. Firefly luciferase activity was normalized to co-transfected renilla luciferase activity. e Bar graph showing amino acid exchange rates in control and WDR4 patient fibroblasts. n = 2. f Bar graph depicting posttranslational modifications in control and WDR4 patient fibroblasts. Inset shows ubiquitination modification. n = 2.