Figure 4.

Pulse-chase mitochondrial translation experiment in control and patient fibroblasts in which the PDF was silenced using siRNA. (A) Suppression of PDF (KD) globally increased the rate of protein synthesis in the pulse by ∼50% in both control and patient cell lines. The migration of individual OXPHOS subunits is indicated on the right of the figure. (B) Immunoblot showing siRNA-mediated silencing of PDF in fibroblasts from controls and MTFMT patients. Serine hydroxymethyltransferase 2 (SHMT2) was used as a loading control.