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. 2020 Feb 28;10:3715. doi: 10.1038/s41598-020-60431-6

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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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Loss of ANG ribonuclease activity correlates with longer ALS duration. Spearman’s Coefficient (p value 0.002) and Kendall Tau’s coefficient (p value 0.005) were used for analyzing correlation. Kaplan-Meier survival analysis is performed and the statistical significance of differences in survival between the categories was evaluated using Log-rank (p value 0.002), Breslow (p value 0.01), and Tarone-ware (p value 0.005) tests. (a) Scatter plot demonstrating significant correlation between ribonuclease activity and ALS onset. Sizes of points are proportional to number of cases. (b) Kaplan-Meier curves illustrating significant differences in disease duration between patients with ANG variants with %WT ribonuclease activity less than or equal to 10% and variants with %WT ribonuclease activity greater than −10%.