Fig. 1: In vivo regenerative potential of Pax3-induced myogenic progenitors in iDUX4pA-HSA mice.
From: Transplantation of PSC-derived myogenic progenitors counteracts disease phenotypes in FSHD mice
a Outline of experimental design. b Representative images show immunostaining for RFP (in red) and Dys (in gray) in non-injured (−CTX) and CTX-injured (+CTX) TA muscles from iDUX4pA-HSA mice that had been transplanted with Pax3-induced mouse ESC-derived myogenic progenitors. Images from PBS-injected TA muscle are shown in the upper panel (-CTX). DAPI stains nuclei (in blue). Scale bar is 50 µm. c Representative images showing immunostaining for RFP (in red), PCM1 (in green) and Dys (in gray) in TA muscles of iDUX4pA-HSA mice that had been injected with PBS (left panel) or cells (right panel) from the +CTX group. DAPI stained nuclei in blue. Arrowheads show RFP +/PCM1 + nuclei. Scale bar is 50 µm. d Representative confocal images from +CTX group show a nucleus positive for RFP and PCM1. Scale bar is 10 µm. e Engraftment quantification based on the number of RFP + /Dys+ and RFP + /Dys + /PCM1 + myofibers. Data are shown as mean ± SEM (n = 6 for PBS, n = 6 for -CTX and n = 4 for +CTX). *p < 0.05 by the Student’s t test.
