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Figure 5 Determination of steady-state mitochondrial transfer ribonucleic acid gene for isoleucine (mt-tRNAIle) levels in solid tissues and cultured cells using high-resolution Northern blots. Small ribonucleic acids (1 µg) were separated through a 13%, 8 mol/l urea denaturing polyacrylamide gel, electroblotted onto membranes and hybridized with radiolabeled probes specific for mt-tRNAIle and mt-tRNALeu(UUR) transcripts as described in Methods: High-resolution Northern blot analysis. Together with the patient samples, each panel shows three appropriate control samples prepared from the identical tissue or cell type. (A) Left ventricle of Patient no. IV-03 (family 1). (B) Skeletal muscle of Patient no. IV-03 (family 1). (C) Left ventricle of Patient no. IV-01 (family 2). (D) Cultured myoblasts of Patient no. IV-03 (family 1). (E) Cultured skin fibroblasts of patient no. IV-03 (family 1; lane 1), Patient no. IV-01 (family 2; lane 2), and Patient no. IV-03 (family 2; lane 3).
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