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. 2020 Jun 3;10:9049. doi: 10.1038/s41598-020-65685-8

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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PF-4708671 treatment prevents PIC enhancement in hG93A-SOD1 motoneurons. (A) PICs were significantly larger in hG93A-SOD1 motoneurons compared to WT, but not in those cells that received PF-4708671. Four out of twenty-four (17%) hG93A-SOD1 motoneurons that received PF-4708671 showed profound hypoexcitability and much smaller PICs than other motoneurons, but no significant change in input conductance (G_in). (B) The ratio of PIC to G_in was taken as a metric of altered excitability. The result showed similar trend and significant changes as in PIC analysis in (A). (C) To further investigate the relationship between PIC and input conductance, we plotted their distributions and fitted the data with a linear equation. Within a comparable input conductance range, the hG93A-SOD1 motoneurons had larger PICs. In contrast, the hG93A-SOD1 motoneurons in animals treated with PF-4708671, had smaller PICs regardless of G_in. Wild-type, WT; 30 mg/kgBW PF-4708671, PF; hypoexcitable, Hypo; persistent inward current, PIC; input conductance, G_in; **P < 0.01.