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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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Generation and characterization of human MPS VII iPSC. (A) β-gluc enzymatic activity (expressed in nmol 4-MU/μg of protein/h) in control and MPS VII patient’s fibroblasts. (B) Representative colonies of control and MPS VII iPSC stained for alkaline phosphatase (AP) and the pluripotency-associated markers SSEA-3, Tra-2-49 and NANOG (all in red) and DAPI (blue); scale bars 200 µm (AP) and 100 µm (SSEA-3, Tra-2-49 and NANOG). (C) Karyotype of MPS VII iPSC. (D) Immunofluorescence microscopy of control and MPS VII iPSC differentiated in vitro and stained for the endoderm, mesoderm and ectoderm markers α-fetoprotein (green), smooth muscle actin (SMA, green) and βIII-tubulin (Tuj1, green), respectively; scale bars 100 µm. (E) Control and MPS VII iPSC differentiated in vivo by teratoma formation, stained with hematoxylin and eosin, showing potential to differentiate into endoderm (intestinal epithelium), mesoderm (cartilage) and ectoderm (neural tube); scale bars 100 µm.