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. 2022 Feb 14;9:820105. doi: 10.3389/fcell.2021.820105

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Copyright © 2022 Sainio, Rasila, Molchanova, Järvilehto, Torregrosa-Muñumer, Harjuhaahto, Pennonen, Huber, Herukka, Haapasalo, Zetterberg, Taira, Palmio, Ylikallio and Tyynismaa.

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NFL protein is not induced to detectable levels by TRID or NMDi treatment in PT iPSC-MN. (A) Immunoblotting series (same blot sequentially analyzed with different antibodies) shows no detectable full-length NFL in treated patient motor neurons. Short and long exposure times shown with two separate NFL antibodies. Treatment between days 16–25 with 25 and 50 µM of amlexanox (AMX), 50 and 100 μg/ml of gentamicin (GEN) and 100 and 200 µM of PTC-124 (PTC). Asterisk indicates an unspecific band detected by the NFL antibody. Concentration as µM for AMX and PTC, and µg/ml for GEN. (B) Representative images of AMX toxicity in day 21 motor neurons (treated for 5 days). Motor neuron health is categorized to—no effect, + some neurite swellings, ++ swellings in most neurites, +++ neurite disruption and ++++ severe neurite disruption and cell death. Higher concentration causes more severe disruption. Scale bar 100 µm.