Figure 6

PNP supplementation reduced intrinsic apoptosis from PNP-deficient induced pluripotent stem cell-derived neurons. The soma (A) and nuclei (B) volume, the average mitochondrial membrane potential (C), cleaved caspase-9 (D), and p53 (E) fluorescence of neurons derived from PNP deficient (PNP−/−) iPSC-derived neurons with or without TAT-PNP replacement enzyme supplemented. The average relative fluorescence units (RFU)/µm3 was calculated by measuring fluorescence throughout the whole volume of a neuron. Data shown as mean + SD of n = 1050 (350 neurons from 3 replicates); *p < 0.001.