Analysis of Patient Mitochondria Reveals Abnormal Crista Membrane Architecture, Related to Figures S1 and S2
(A and C) Representative fibroblasts from control and patient were stained with MitoTracker Green FM and imaged by live confocal fluorescence microscopy at a magnification of 63×, showing typical morphology of mitochondria. Scale bars represent 5 μm.
(B and D) Control and patient mitochondria were imaged by in situ cryo-electron tomography at a nominal magnification of 15,500×, and three-dimensional models of each organelle were generated by manual segmentation. Key mitochondrial structural features are indicated, including: mitochondrial outer membrane (MOM), intracristal space (ICS), mitochondrial inner membrane (MIM), and matrix granules (MG), with three-dimensional reconstructions at right. Scale bars represent 200 nm.