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 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.
MeSH terms
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Amino Acid Transport Systems, Basic / genetics
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Amino Acid Transport Systems, Neutral / genetics
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Animals
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Cystine / drug effects*
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Cystine / metabolism
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Cystinuria / metabolism*
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Disease Models, Animal
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Kidney / diagnostic imaging
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Kidney / drug effects*
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Kidney / metabolism
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Mice
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Mice, Knockout
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Solubility / drug effects
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Thioctic Acid / pharmacology*
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Urolithiasis / diagnostic imaging
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Urolithiasis / metabolism*
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X-Ray Microtomography
Substances
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Amino Acid Transport Systems, Basic
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Amino Acid Transport Systems, Neutral
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Slc3a1 protein, mouse
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Cystine
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Thioctic Acid