Chapter 2 42 (1:200, Thermo Fisher Scientific, Waltham, MA USA). Megalin fluorescence intensity measurements were performed using ImageJ software. The region of interest was selected outlining the periphery of the kidney tubule, and background fluorescence was set at the same intensity as the internal lumen, and subtracted from the total fluorescence intensity38. Statistical analysis Statistical analysis was performed using WINPEPI statistical software, version 11.4363. Unless otherwise specified, results were expressed as mean ± standard deviation and differences were tested using the student′s unpaired t test for numerical data and by Chisquared test for categorical data. Two-tailed P values <0.05 were considered significant. Acknowledgments M.A.E is supported by ERA-Net, E-Rare2-JTC2014: Novel therapies for cystinosis. E.L is supported by the Research Foundation - Flanders (F.W.O. Vlaanderen), grant 1801110N and the Cystinosis Research Network (CRN). We gratefully acknowledge Francesco Emma (Bambino Gesù Children’s Hospital, IRCCS, Rome) for valuable discussions and scientific assistance and Pieter Baatsen (VIB BIO Imaging Core, KU Leuven) and Tobias Starborg (University of Manchester FMBH EM Facility) for help with the development of EM images. We sincerely thank Marina G. Mori da Cunha, Ekaterina A. Ivanova, Inge Bongaers, Daniëlle Copmans and Jan Maes (KU Leuven) for valuable assistance. Author contributions M.A.E, LV and E.L designed the experiments. M.A.E, R.K, L.K, L.K, F.A.O, J.M, A.P, P.T, A.N, P.A.D, M.L, H.J.B, L.V and E.L performed the experiments and/or analyzed the results and interpreted the data. M.A.E and R.K drafted the manuscript and all authors critically revised the manuscript for intellectual content. Competing financial interests The authors declare no competing financial interests.
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