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. 2019 Jul 31;13:356. doi: 10.3389/fncel.2019.00356

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Copyright © 2019 La Rosa, Russo, D’Amico, Petrillo, Aquilano, Lettieri-Barbato, Turchi, Bertini and Piemonte.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Frataxin depletion determines impairments in NRF-2 expression and signaling. (A) ROS determination (graph on the left) and Complex I activity (graph on the right), assessed in WT and KIKO NSCs. Cellular ROS were evaluated by measuring DCF fluorescence, normalized for cell number, while Complex I activity was expressed as nmol/min/mg prot. qPCR (B) and Western Blot analyses (C) and relative densitometric evaluation (graph on the right) of the expression of the transcription factor Nrf2, its targets (NQO1 and HO-1) and Frataxin, in WT and KIKO NSCs. GAPDH was used for qPCR normalization, while Tubulin was used as Western Blot loading control, ^*p < 0.05 and ^∗∗p < 0.01.