Fig. 6: FIB/SEM micrographs show abnormal ultrastructural features of RTD MNs.
From: Caspase-dependent apoptosis in Riboflavin Transporter Deficiency iPSCs and derived motor neurons
(A column) Electron micrographs show abnormal nuclear morphology in RTD MNs, characterized by an irregular shape, often lobed. (A column, arrow) The presence of chromatin clumps is reminiscent of apoptotic process. (B, C columns) Ctrl MNs show healthy mitochondria with well-developed cristae while in RTD MNs several damaged organelles with either dilated (asterisks) or disrupted (arrowheads) cristae are observed. Patients’ MNs show a significantly higher number of damaged mitochondria with severe or mild phenotype, compared to Ctrl cells that have a higher percentage of healthy organelles. The percentages of mitochondria with mild or severe phenotype were obtained by manually counting and classifying mitochondria of Ctrl and RTD MNs. Data are presented as the mean ± SEM and analyzed by T tests. ****p ≤ 0.0001; ***p ≤ 0.001; **p ≤ 0.01; *p ≤ 0.05. A Scale bars, 2 µm; (B, C) Scale bars, 1 µm.
