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. 2018 Nov 9;8:16644. doi: 10.1038/s41598-018-34523-3

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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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MPS VII neural cells recapitulate known disease features. (A) β-gluc enzymatic activity (expressed in nmol 4-MU/μg of protein/h) in control and MPS VII neurons, non-treated (N.T.) and treated with rβ-gluc. (B) β-gluc enzymatic activity in control (black) and MPS VII (grey) neurospheroids at day 7 and 28. (C) Gene expression analysis of control (black) and MPS VII (grey) neurospheroids; fold-change (normalized to undifferentiated cells) of GUSB. (D) GAGs storage in control and MPS VII neurons by toluidine blue staining at 6 and 9 weeks; scale bars 5 μm. (E) Number of toluidine blue nonstaining vesicles per cell at week 9; micrographs were analyzed using ImageJ software and the number of nonstaining vesicles/cell, with a minimum of 0.5 and a maximum of 4.2 μm, were counted manually by a masked observer; each data point (n) corresponds to a single, well isolated, cell (control n = 20, MPS VII n = 23). (F) GAGs quantification in control (black) and MPS VII (grey) neurospheroids by Blyscan Sulphated Glycosaminoglycan assay. (G) β-hex enzymatic activity (expressed in nmol 4-MU/μg of protein/h) in control (black) and MPS VII (grey) neurospheroids at day 7 and 28. Data are mean ± SD of 3 (control) and 4 (MPS VII; 2 with MPS VII-cl.8 and 2 with MPS VII-cl.13) independent cultures. ***p < 0.001.