Fig. 7
Downregulated miR-18b (miR-18b-5p) by SOD1 mutations is associated with apoptotic cell death in motor neuron from hiPSC-derived NSCs fALS SOD1 (G17S). a Nanog (green) and Oct4 (red) iPSC marker are expressed in hiPSCs (normal and fALS SOD1 (G17S). Scale bar, 20 μm. b Neural stem cells (SOX2 and Nestin) was generated from hiPSCs (normal and fALS SOD1 (G17S)). Scale bar, 40 μm. c Differentiated motor neurons from hNSCs were double-stained with HB9 and ChAT antibodies. Scale bar, 20 μm. d Hif1α and Mef2c transcripts were upregulated in differentiated motor neurons SOD1 (G17S). e The mRNAs levels of Mctp1 and Rarb was downregulated in differentiated motor neurons SOD1 (G17S). f Intracellular Ca2+ levels (Normal (0.149) versus SOD1 (G17S) (0.215) in fluorescence intensities from baseline 490/525 ratio) were enhanced in differentiated motor neurons SOD1 (G17S). Total neurite length was reduced in differentiated motor neurons SOD1 (G17S). g miR-18b (miR-18b-5p) expression was deeply low and mir-206 was highly expression in differentiated motor neurons SOD1 (G17S). Significantly different at *, p < 0.05; **, p < 0.005. The experiments were replicated 5 times