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α-Lipoic acid treatment prevents cystine urolithiasis in a mouse model of cystinuria
- Zee, Tiffany;
- Bose, Neelanjan;
- Zee, Jarcy;
- Beck, Jennifer N;
- Yang, See;
- Parihar, Jaspreet;
- Yang, Min;
- Damodar, Sruthi;
- Hall, David;
- O'Leary, Monique N;
- Ramanathan, Arvind;
- Gerona, Roy R;
- Killilea, David W;
- Chi, Thomas;
- Tischfield, Jay;
- Sahota, Amrik;
- Kahn, Arnold;
- Stoller, Marshall L;
- Kapahi, Pankaj
- et al.
Published Web Location
https://doi.org/10.1038/nm.4280Abstract
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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