1. Global defects to mitochondrial protein synthesis remodels cristae ultrastructure.
Global defects to mitochondrial protein synthesis remodels cristae ultrastructure. (A) Representative transmission electron micrographs of human fibroblasts treated with the indicated siRNAs with or without chloramphenicol (CA). (B) Representative transmission electron micrographs of human myoblasts with the same nuclear genetic background that are either homoplasmic for wild-type mitochondrial DNA (mtDNA) or the m.8344A>G tRNALys mutation. (C) Representative transmission electron micrographs of human fibroblasts from a patient with a pathogenic mutation in C12orf65 stably transduced with an empty vector or the wild-type C12orf65 cDNA. Bottom right, immunoblotting of whole-cell lysates with the indicated antibodies. (D) Representative transmission electron micrographs of human fibroblasts with the MT-ATP6 m.9205delTA mutation heat shocked for 4 h at 45°C. (E) Scatter plot quantification of mitochondrial morphology under light microscopy in human fibroblasts with the MT-ATP6 m.9205delTA mutation grown at 37°C or heat shocked for 4 h at 45°C with or without CA. Data are from three independent experiments. SC, scrambled sequence.
Uwe Richter et al. LSA 2019;2:e
© 2019 Richter et al.