Impaired myogenic development, differentiation and function in hESC-derived SMA myoblasts and myotubes
Fig 5
Reduced cholinergic Ca2+ response in differentiated myotubes from SMA patients.
RT-qPCR of relative AChR subunit expression CHRNA1 (A) and CHRNG (B) decreased in SMA Type 1 cells. Fluorometric measurement of intracellular Ca2+ concentrations changes by carbachol treatment in ESC-SkM. Dose-response curve carbachol responses for WT and SMA ESC-SkM showed different sensitivity between genotypes (EC50 values, p < 0:05, sum-of-squares F-test). Baseline-subtracted amplitudes of carbachol responses normalized to baseline subtracted amplitudes of 4-Br-A23187 responses. These ratios were normalized to maximal values within an experiment. Data represented as mean ± SEM (A, B) and mean ± SEM of n = 6–8 individual samples from two independent experiments. *: p < 0:05, **: p < 0:01, ***: p < 0:001, two-tailed unpaired Student's t-test.