Fig. 6

Defects in [Ca²⁺]_i handling are recapitulated in cardiomyocytes derived from SMA patient iPSCs. a Representative [Ca²⁺]_i transients measured in iPSC-derived cardiomyocytes engineered from unaffected control (NCRM1) and SMA patient (GM23240) iPSCs illustrating the slowed removal of cytoplasmic Ca²⁺. b Expression of SERCA2a expression is increased (western blot, left) and T₅₀ is decreased (right) in SMA patient iPSC-derived cardiomyocytes when SMN protein is restored. Cells were transfected on day 18 of differentiation and recordings were made on day 20. c Similarly, expression of SERCA2a expression is reduced (western blot, left) and T₅₀ is increased (right) in unaffected cotnrol iPSC-derived cardiomyocytes when SMN protein is reduced. Cells were transfected on day 18 of differentiation and recordings were made on day 20. Statistical analysis: p values shown for one-way ANOVA, Tukey’s post hoc test, n = 7