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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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Effects of PF-4608671 administration of motoneuron size. (A) Motoneuron volume is significantly larger in hG93A-SOD1 group compared to wild-type control. The administration of PF-4608671 significantly reduced motoneuron soma volume in hG93A-SOD1. (*P < 0.05; **P < 0.01; WT, N = 120/24 [# of MNs/animals]; mSOD1, N = 60/12; WT + PF, N = 75/15; mSOD1+PF, N = 76/15). (B) The soma volume measures are binned every 5,000 μm³. The percentage is calculated as: (number of motoneurons in specific bin/number of total motoneurons in specific group). Increased frequency in large motoneurons and decreased frequency in medium motoneurons with less affected small motoneurons in hG93A-SOD1 group indicate the increase in volume of certain medium motoneurons. The application of PF-4708671 successfully restored the two-peak pattern in hG93A-SOD1 back to single peak as wild-type. Wild-type, WT; hG93A-SOD1, mSOD1; PF-4708671, PF; MN, motoneuron.