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. 2018 Oct 15;8:15229. doi: 10.1038/s41598-018-33449-0

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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/.

PMC Copyright notice

Identified CLN2 disease-specific metabolite signatures linearly correlate with CLN2 disease severity scores. Final Pearson correlation analysis and linear regression analysis of CLN2 specific metabolite signatures and the magnetic resonance imaging disease severity score (MRIDSS) or the Weill Cornell LINCL score (WCLS) of all CLN2 subjects (test and validation cohort). Relative metabolite abundances (ion counts, y-axis) are plotted against the disease severity scores (x-axis). Dotted lines represent the 95% confidence bands. Pearson r and p-values are summarized in Table 2.