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


ABSTRACT: 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 ?-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.

SUBMITTER: Zee T 

PROVIDER: S-EPMC5656064 | biostudies-literature | 2017 Mar

REPOSITORIES: biostudies-literature

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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<sup>-/-</sup> mouse model of cystinuria by increasing the solubility of urinary cystine. These findings id  ...[more]

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