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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/.

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Unknown metabolites 1–6 differentiate CLN2 disease subjects from normal controls. (a–g) MS signal abundances of unknown metabolites 1–6 are decreased in the CLN2 disease group versus normal controls (p < 0.05, see Table 1 for details). Indicated signal abundances were quantified as areas under their cognate chromatographic peaks. h, Linear correlation of MS signal abundances of Unknown 4 with a co-eluting metabolite feature of 139.981 Da (Pearson correlation r > 0.85, p < 0.0001), indicating a relationship as a precursor and in-source fragment ion.