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. 2022 Jul 6;149(20):dev199914. doi: 10.1242/dev.199914

Fig. 6.

Fig. 6.

Leigh syndrome patient-derived organoids show defects in mitochondrial morphology in the sVZ compartment. (A) Representative confocal images of day 30 brain organoids showing mitochondrial morphology (TOM20). The red line divides the Sox2+ NPCs surrounding the lumen (L) from newly committed neurons. MT-ATP6/PDH mutant organoids showed disorganization and fragmentation of the mitochondrial network. (B) Quantification of average mitochondrial volume, diameter, surface area, and major axis length are shown. Graphs represent mean±s.e.m. from at least three independent subventricular zones (sVZs) per phenotype from three independent organoid batches. Quantification was performed by three-dimensional reconstruction of the mitochondrial network of interest. (C) Representative super-resolution images of mitochondrial morphology in LS and control NPCs. (D) Quantification of average mitochondrial number, volume, mitochondrial sphericity and mitochondrial branching are shown. Graphs represent mean±s.e.m. from at least three independent experiments (n>20 cells per genotype). *P<0.05; **P<0.01; ****P<0.0001 (one-way ANOVA). Scale bars: 10 μm (A); 5 μm (C).