Figure 5.

Generalized decrease in mitochondrial translation and in the levels of mitochondrial mRNAs in control and LSFC fibroblasts after LRPPRC knockdown. Fibroblasts from one control and one LSFC patient were transiently transfected either with one of two different siRNA constructs specific to LRPPRC (S1173 and S3017) or with a fluorescent control siRNA (Alexa). On day 6, transfected and untransfected cells were either pulse-labeled with [³⁵S]methionine and cysteine (A) or total RNA was extracted and analyzed by Northern blot (B). (A) The seven subunits of complex I (ND), one subunit of complex III (cyt b), three subunits of complex IV (COX), and two subunits of complex V (ATP) are indicated at the left of the figure. (B) Hybridization was performed with probes specific for the mitochondrial mRNAs encoding the three COX subunits, the ND1 subunit of complex I, and the bicistronic mRNA encoding the complex V subunits (ATP6/8) and with probes for the 12S and 16S mitochondrial rRNAs.