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. 2018 Apr 19;8:6247. doi: 10.1038/s41598-018-24717-0

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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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Intracellular localization of ATP7B in HLCs obtained from control and patient iPSCs. (a) Confocal immunofluorescence analysis of HLCs immunolabeled for ATP7B and Golgin 97 (upper panels) or KDEL containing proteins (lower panels) as markers of Golgi complex and ER, respectively. White arrows point to Golgi complex containing ATP7B-H1069Q in patient HLCs. Scale bars: 10 µm. (b) Immuno-electron microscopy images demonstrate distribution of ATP7B in control and patient cells. ATP7B was preferentially localized at the Golgi (white arrows) in control cells. Both cisternae of the ER (black arrows) and the Golgi membranes (white arrows) contained ATP7B in cells from patient. Scale bars: 300 nm. Graph represents % of ATP7B-associated gold particles (means ± SD) counted within ER and Golgi compartments in control and patient cells.