Cystinuria is an incompletely dominant disorder characterized by defective urinary cystine reabsorption that results in the formation of cystine-based urinary stones. Current treatment options are limited in their effectiveness at preventing stone recurrence and are often poorly tolerated. We report that the nutritional supplement α-lipoic acid inhibits cystine stone formation in the Slc3a1−/− mouse model of cystinuria by increasing the solubility of urinary cystine. These findings identify a novel therapeutic strategy for the clinical treatment of cystinuria.
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We thank R. Murphy and S. Melov for their advice regarding μCT; T. Te Koi for histology assistance; M. Chamoli and N. Mathew for help with imaging; and D. Chrzan for helpful discussions. This work was supported by grants from the American Federation of Aging Research (to P.K.), the Larry L. Hillblom Foundation (to P.K.), the Boston Scientific Foundation (to M.L.S.), and the NIH (R01 AG038688 and R01 AG045835 to P.K.; R21 DK091727 to P.K. and M.L.S.; P20 DK100863 and R21 DE025961 to M.L.S.; K12 DK083021 and R21 DK109433 to T.C.).
The authors declare no competing financial interests.
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Zee, T., Bose, N., Zee, J. et al. α-Lipoic acid treatment prevents cystine urolithiasis in a mouse model of cystinuria. Nat Med 23, 288–290 (2017). https://doi.org/10.1038/nm.4280
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