1. Heat shock during translation of an aberrant mitochondrial mRNA activates OMA1 and a ribosome decay pathway.
Heat shock during translation of an aberrant mitochondrial mRNA activates OMA1 and a ribosome decay pathway. (A) Immunoblotting of whole-cell lysates from the MT-ATP6 m.9205delTA fibroblasts. (B) Left, immunoblotting of whole-cell lysates from the MT-ATP6 m.9205delTA fibroblasts. Right, quantification of the immunoblotting from three independent experiments. Data represent mean ± SD. (C) Left, representative images of Northern blotting of total cellular RNA from MT-ATP6 m.9205delTA fibroblasts. Right, quantification of the Northern blotting from four independent experiments. Data represent mean ± SD. (D) A representative image of Southern blotting for mitochondrial DNA copy number from the MT-ATP6 m.9205delTA fibroblasts from multiple independent experiments. (E) Quantitative label-free liquid chromatography–mass spectrometry/mass spectrometry analysis of mitochondrial ribosomal subunits from the large (LSU) and small (SSU) subunit of wild-type and MT-ATP6 m.9205delTA fibroblasts grown at 37°C or heat shocked for 4 h at 45°C. Each data point represents a single mitochondrial ribosomal protein. Data were collected from isolated mitochondria from five independent experiments for each genotype and temperature condition. (F) Immunoblotting of whole-cell lysates of human fibroblasts with the indicated MT-ATP6 genotypes following culture at 37°C or heat shocked for the indicated time at 45°C treated with the indicated translation inhibitors. Anisomycin inhibits translation elongation on cytoplasmic ribosomes.
Uwe Richter et al. LSA 2019;2:e
© 2019 Richter et al.