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. 2023 Feb;193(2):201–212. doi: 10.1016/j.ajpath.2022.11.002

Figure 4.

Figure 4

POLG mutation decreases mitochondrial content in induced pluripotent stem cell–derived cortical neurons. A: Representative fluorescence microscopy images of 8-week–old neurons expressing green fluorescent protein (GFP) and mtDsRed. B: Quantification of mitochondrial content in neurites, expressed as a percentage of neurite length occupied by mitochondria. C: Western blot analysis of lysate from neurons using antibodies for nuclear-encoded protein succinate dehydrogenase A (SDH-A), mitochondrial-encoded protein cytochrome c oxidase 1 (COX-I), and β-actin. D: Quantification of COX-I, with protein levels expressed as a ratio to β-actin, normalized to the mean of both control lines. E: Quantification of SDH-A with protein levels expressed as a ratio to β-actin. Data are expressed as means ± SD. n = 13 neurons from three independent differentiation experiments (B); n = 5 differentiation experiments (D and E). PgATWS versus PgATLX, P = 0.057 (B). Analysis of variance, P = 0.0499, F (DFc, DFd) 3.242 (3, 16), post hoc O2SF versus PgATWS, P = 0.053 (E). ∗∗P < 0.01, ∗∗∗P < 0.001, ∗∗∗∗P < 0.0001. Scale bars = 10 μm (A).