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. 2018 Apr 16;8:6030. doi: 10.1038/s41598-018-24463-3

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© The Author(s) 2018

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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3B12A scFv prevents the growth and proliferation of cytoplasmic TDP-43 aggregates to reduce cell death. (a) Time-lapse imaging of cytoplasmic TDP-43 aggregates in the presence or absence of 3B12A scFv-CMA in HEK293A cells. Scale bar = 100 µm. (b–d) Quantified analysis of time-lapse imaging. TDP-43 aggregates were distinguished from non-aggregates by diminishing the fluorescence intensity of diffusely distributed cytoplasmic GFP-TDP-43 signals and subsequently quantified with ImageJ software. Differences were evaluated by two-way ANOVA (mean ± SD from three independent visual fields; *p < 0.05, ***p < 0.005 and ****p < 0.001). (e,f) Effects of TDP-43 species and 3B12A scFv on N2a cell toxicity (e) and viability (f) DCS represents the C173S/C175S mutant. Differences were evaluated by one-way ANOVA (mean ± SD from triplicate; *p < 0.05, **p < 0.01, ***p < 0.005 and ****p < 0.001). (g) R110/AFC ratio obtained from (e,f) Differences were evaluated by one-way ANOVA (mean ± SD from triplicate; ***p < 0.005 and ****p < 0.001). N.S. indicates not significant.