Figure 5.

Lentivirus mediated expression of wild-type MRPS25 rescues the mt-SSU and OXPHOS levels in patient cells. Analysis of the small (MRPS25, MRPS17, MRPS27, MRPS22) and large (MRPL45) mitochondrial subunits (A) and OXPHOS components of complex I (NDUFB8), complex III (UQCRC2), complex IV (COXI) (B) in immortalized cell extracts from control (C1 and C2), patient (P) and patient transduced with wild-type MRPS25 (P^S25). Vinculin (VCL) was used as a loading control. To the right of A and B, relative abundance of the ribosomal protein and OXPHOS components in untransduced versus the transduced fibroblasts (Fiji ImageJ densitometric analysis). The data are shown as the mean ± standard error of the means of n ≥ 3 independent experiments. Probability was determined using Welch’s t-test (ns = not significant, P > 0.05; ^*P < 0.05).