Impaired myogenic development, differentiation and function in hESC-derived SMA myoblasts and myotubes
Fig 7
Reduced ATP production in myotubes from SMA patients.
ATP levels were reduced in myotubes of SMA and unaffected cells compared to myoblasts (A). Challenge with Oligomycin and treatment with Cyclosporin A in myoblasts increased ATP levels only in SMA Type 1 derived myoblasts (B), while in myotubes Oligomycin challenge decreased in all cells the ATP levels, but also here Cyclosporin A had only a protective effect on SMA Type 1 cells (C). Mitochondrial DNA levels, an indicator for the number of mitochondrial, were not significantly reduced in SMA derived cells (D). Data represented as mean ± SEM of n = 3 individual samples. p < 0:05, **: p < 0:01, ***: p < 0:001, two-tailed unpaired Student's t-test, (A-D n = 3).