α-Lipoic acid treatment prevents cystine urolithiasis in a mouse model of cystinuria

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 often poorly tolerated. We report tha...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2017
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5656064/
https://www.ncbi.nlm.nih.gov/pubmed/28165480
http://dx.doi.org/10.1038/nm.4280
Descripción
Sumario: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 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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