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. 2013 Mar 5;8(3):e57631. doi: 10.1371/journal.pone.0057631

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© 2013 Young-Gqamana et al

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.

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Eight-week old male Fabry hR301Q α-Gal A Tg/KO mice were administered either water or migalastat HCl (100 mg/kg ad libitum in drinking water) daily or less frequently (4 on/3 off) for 28 days. Kidney, heart, and skin were subsequently harvested and analyzed for (A) lyso-Gb₃ and (B) GL-3 levels. The lyso-Gb₃ and GL-3 data represent the mean ± SEM of 10 mice/group. *p<0.05 compared to control; #p<0.05 compared to daily; t-test.