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. 2020 Jul 17;17:213. doi: 10.1186/s12974-020-01866-6

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© The Author(s) 2020

Open AccessThis 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 licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence 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 licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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Fig. 2 — Behavioral analysis revealed significant reduction in motor function over time in Nlrp3^L351P cohort. Behavioral analyses were conducted at 6, 10, 14, and 18-month time points in cohorts (Nlrp3^WTn = 11, Nlrp3^A350Vn = 6, Nlrp3^L351Pn = 9). aNlrp3^L351P mice had significant rotarod behavior deficits with age (two-way ANOVA, p = 0.0211 (genotype), p = 0.0052 (time); Tukey’s post hoc multiple comparisons test, * p = 0.02). b, c Endpoint analysis at 18 months shows Nlrp3^L351P expressing mice, not Nlrp3^A350V, were significantly impaired compared to Nlrp3^WT (* p = 0.026, t test). In open field analysis, animals expressing Nlrp3^L351Pd moved for significantly less time (two-way ANOVA, p = 0.0018 (genotype), p < 0.0001 (time); Tukey’s post hoc multiple comparisons test, ** p = 0.0027), g moved a significantly shorter distance (two-way ANOVA, p = 0.0008 (genotype), p < 0.0001 (time); Tukey’s post hoc multiple comparisons test, ** p = 0.0015), and j traveled at a significantly lower velocity (two-way ANOVA, p = 0.0008 (genotype), p < 0.0001 (time); Tukey’s post hoc multiple comparisons test, ** p = 0.0020) at 18 months compared to 6 months of age. No significant changes were observed in the Nlrp3^WT and Nlrp3^A350V over time. Endpoint comparison at 18 months between cohorts revealed a significant decline in f move time (* p = 0.0135, t test), i distance moved (** p = 0.0085, t test), and l total velocity (* p = 0.01, t test) in animals expressing Nlrp3^L351P compared to Nlrp3^WT animals. e, h, k This difference was not observed in Nlrp3^A350V expressing animals when compared to controls. Error bars represent s.e.m.