Fig. 5: TANGO2 is required for intermediate filament structure that is used by the mitochondrial network.

a Three dimensional renders of mitochondria detected in the control and TANGO2^–/– cells. For each cell, a full fluorescence micrograph, inset of the region isolated for FIB-SEM imaging, and the maximum value projection of raw AIVE data are shown; the reconstructed mitochondria and their distribution from the FIB-SEM dataset are shown. Scale bars, 2 μm (fluorescence micrographs) and 1 μm (fluorescence micrograph insets, electron micrographs and raw AIVE data); each notch around the reconstructed datasets represents 1 μm. The positive and negative curvature of mitochondria are shown in red and blue, respectively. b Quantitation of the mean curvature of the mitochondrial exterior. Values are means ± SD, *p < 0.05, **p < 0.01, Student’s two-tailed t test (n = 40). c Sum projected images of 3D voxel values showing surface filopodia detected in the control and TANGO2^–/– cells. d Immunostaining of control and TANGO2 patient fibroblasts with desmin (green) and TANGO2 (red) antibody. The results are representative of three independent biological samples. e Immunoblots of control and TANGO2 patient fibroblasts probed with desmin, vimentin, TANGO2 and CRYAB. GAPDH was used as loading control (n = 3 biological replicates). All values are means ± SD *p < 0.05, **p < 0.01, ***p < 0.001, Student’s two-tailed t test. Source data are provided as a Source Data file.