FIGURE 6.

Mutant CPCs have reduced levels of both nuclear and mitochondrial encoded OXPHOS proteins and reduced activation of mitochondrial biogenesis. A, representative Western blots revealed reduced levels of selective OXPHOS subunits in POLG CPCs. B, quantitation of mitochondria-encoded complex IV Subunit 1 (n = 5) and nuclear-encoded subunit 4 protein expression (n = 5). C, real-time qPCR analysis of Cox1 (complex IV Subunit 1) and Cox4i1 (complex IV subunit 4) gene expression at baseline (n = 3). D, real-time qPCR analysis of PGC-1α (n = 3) and PGC-1β (n = 3) gene expression at day 0 and day 7 in WT and POLG CPCs. *, p < 0.05; **, p < 0.01; ***, p < 0.001; n.s., not significant.