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. 2017 Mar 30;292(21):8907–8917. doi: 10.1074/jbc.M116.762898

Figure 4.

Figure 4.

A, i, transmission electron microscopy images of mitochondria within control and mutant iPSC-derived neurons at day 80 of differentiation. Mutant neurons consistently display a small proportion of swollen, electron dense mitochondria. Arrows show damaged cristae. Scale bar represent 1 μm. ii, quantification of swollen mitochondria. n = 3 control, n = 3 R155C and n = 1 R191Q. B, representative image of a neuron transfected with mitAT1.03 probe. C, representative traces of mitochondrial ATP after ADP application. Oligomycin was applied as a negative control. Scale bar represent 10 μm. D, quantification of the rate of ATP increase in control and VCP mutation bearing iPS cells. E, quantification of the rate of ATP increase in control and VCP iPS-derived neurons. *, p < 0.05; *, p < 0.05; ***, p < 0.001.