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Fig. 1 | Stem Cell Research & Therapy

Fig. 1

From: DP71 and SERCA2 alteration in human neurons of a Duchenne muscular dystrophy patient

Fig. 1

ac Semithin CTRL section stained with toluidine blue (a) showing a rounded neurosphere with healthy cells, and ultrathin section (b, c) of hiPSCs showing a larger CTRL hiPSCs with abundant mitochondria and organelles (b) compared to DMD hiPSCs with heterocromatic and irregular nucleus (c). di Dp71 green and AQP4 red confocal immunofluorescence show an intensity fluorescence reduction in DMD hiPSCs (gi) compared to the control ones (df). Dp71 green signal is membrane disarranged and scattered in the cytoplasm in DMD hiPSCs (h arrow). An orange fluorescence colocalization of Dp71 and AQP4 is present in control cells (d yellow arrow) while separate fluorescent signals are present in DMD cells (g green and red arrows). lq Dys red and βDG green confocal immunofluorescence display a Dys pointed cytoplasm signals (o, p red arrow) and disarranged βDG green fluorescent in DMD hiPSCs (o, q green arrow) compared to strong and polarized signals in the controls (ln red arrow, green arrow). A merge orange signal for Dys and βDG is present in short membrane tracts in CTRL hiPSCs (l orange arrowhead) while separate signals are present in DMD hiPSCs (o green and red arrows). Morphometric analysis (r) shows a significant Dp71, AQP4, Dys, and βDG fluorescence intensity reduction in DMD compared to CTRL cells. The mRNA expression analysis (s) shows reduction of Dp71, AQP4, Dys mRNA, and increased of DG mRNA in the DMD cells compared with control. Scale bar: a 30 μm; b, c 2.5 μm; dq 7.5 μm. Data are represented as mean ± SEM

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