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. 2017 Jul 24;7:6320. doi: 10.1038/s41598-017-06264-2

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© The Author(s) 2017

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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Representative ¹H CPMG NMR spectrum of (a) untreated NPC1 patient plasma with 27 major metabolites labelled; (1) mobile lipid -CH₃, (2) isoleucine, (3) leucine, (4) valine, (5) 2,3 butanediol, (6) 3-hydroxybutyrate, (47) mobile lipid -(CH₂)_n-, (8) lactate, (9) alanine, (10) mobile lipid –CH ₂CH₂CO, (11) arginine, (12) acetate, (13) proline/mobile lipid CH₂-CH ₂-CH=, (14) N-acetyl glycoprotein/mobile lipid CH₂-CH ₂-CH=, (15) glutamate, (16) glutamine, (17) acetoacetate, (18) citrate,(19) Ca²⁺-EDTA, (20) Mg²⁺-EDTA, (21) EDTA, (22) Zn²⁺-EDTA, (22) α-glucose, (23) mobile unsaturated lipids >CH=CH<, (24) tyrosine, (25) histidine, (26) phenylalanine, (27) formate, and (H) contaminants arising from histopaque cell separation. (b) ¹H NMR spectrum of histopaque column flow through; matching contaminants are clearly visible in the plasma spectrum.