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Figure 4 CHCHD2 but not TOP1MT expression rescues molecular defects
CHCHD2 but not TOP1MT expression rescues molecular defects (A) Mitochondrial morphology of control and patient fibroblasts, in the presence or absence of either wild-type CHCHD2 or wild-type TOP1MT, was examined using MitoTracker Orange and visualized by fluorescence microscopy. The scale bar represents 10 µm. Arrows indicate refractile granules characteristic of network fragmentation. (B) Oxygen consumption of respiratory complexes was measured in control, patient fibroblasts, and patient fibroblasts expressing either wild-type CHCHD2 or wild-type TOP1MT using an OROBOROS respirometer. Data are presented as mean ± SEM and were analyzed using a Student's t test. *p < 0.05, **p < 0.01. Richard G. Lee et al. Neurol Genet 2018;4:e276 Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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