Fig. 4
Human dysferlin-mutated myotubes present connexins 40.1, 43 and 45. Human dysferlin-mutated myotubes were obtained by differentiation of dysferlin-mutated myoblast lines named 107, 379, AB320 and ER, each one bearing different dysferlin mutations. Connexins 40.1, 43 and 45 were detected by immunofluorescence studies using specific antibodies. All cell lines showed positive reactivity to Cxs (green signal) compared with control myotubes (RCMH), where Cxs reactivity was not detected. P2X7 receptor and TRPV2 channel were also detected by immunofluorescence studies using specific antibodies. Only 107 and 379 myotubes expressed P2X7 receptors and TRPV2 channels. Nuclei were stained with DAPI (blue signal). Scale bar: 30 μm. n = 4 cell cultures for each cell line