Fig. 4.

Aminoacylation status of mitochondrial tRNAs.

Northern blot analysis of mitochondrial tRNA aminoacylation in total RNA samples from proband 1 (P1) and 2 (P2), and control fibroblasts (C1, C2). Membranes were hybridized with radioactive mt-tRNA probes as indicated. “dAc” indicates deacylated control sample. Densitometric analysis of the northern blot hybridized with mt-tRNA Phe shows a decrease of the ratio between aminoacylated (aa-) and deacylated form, as compared to controls. This decrease is more distinct in proband 2. A slight difference in the migration of the two mt-tRNASer products is also seen in proband 2.