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 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.
Department of Urology, University of California, San Francisco, San Francisco, California, USA.|Buck Institute for Research on Aging, Novato, California, USA.
Tiffany
T
Zee
Department of Urology, University of California, San Francisco, San Francisco, California, USA.|Buck Institute for Research on Aging, Novato, California, USA.
Neelanjan
http://orcid.org/0000-0002-3817-4757
N
Bose
Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA.
Jarcy
J
Zee
Department of Urology, University of California, San Francisco, San Francisco, California, USA.|Buck Institute for Research on Aging, Novato, California, USA.
Jennifer N
JN
Beck
Buck Institute for Research on Aging, Novato, California, USA.
See
S
Yang
Division of Urology, Robert Wood Johnson Medical School, Rutgers University, New Brunswick, New Jersey, USA.
Jaspreet
J
Parihar
Department of Genetics and the Human Genetics Institute of New Jersey, Rutgers University, Piscataway, New Jersey, USA.
Min
M
Yang
Buck Institute for Research on Aging, Novato, California, USA.
Sruthi
S
Damodar
Buck Institute for Research on Aging, Novato, California, USA.
David
D
Hall
Buck Institute for Research on Aging, Novato, California, USA.
Monique N
MN
O'Leary
Buck Institute for Research on Aging, Novato, California, USA.
Arvind
A
Ramanathan
Department of Obstetrics, Gynecology, and Reproductive Sciences, University of California, San Francisco, San Francisco, California, USA.
Roy R
RR
Gerona
Department of Urology, University of California, San Francisco, San Francisco, California, USA.|Nutrition and Metabolism Center, Children's Hospital of Oakland Research Institute, Oakland, California, USA.
David W
DW
Killilea
Department of Urology, University of California, San Francisco, San Francisco, California, USA.
Thomas
T
Chi
Department of Genetics and the Human Genetics Institute of New Jersey, Rutgers University, Piscataway, New Jersey, USA.
Jay
J
Tischfield
Department of Genetics and the Human Genetics Institute of New Jersey, Rutgers University, Piscataway, New Jersey, USA.
Amrik
A
Sahota
Buck Institute for Research on Aging, Novato, California, USA.
Arnold
A
Kahn
Department of Urology, University of California, San Francisco, San Francisco, California, USA.
Marshall L
ML
Stoller
Department of Urology, University of California, San Francisco, San Francisco, California, USA.|Buck Institute for Research on Aging, Novato, California, USA.
Pankaj
P
Kapahi
D026923:Amino Acid Transport Systems, Basic / Q000235:genetics; D026921:Amino Acid Transport Systems, Neutral / Q000235:genetics; D000818:Animals; D003553:Cystine / Q000187:drug effects* / Q000378:metabolism; D003555:Cystinuria / Q000378:metabolism*; D004195:Disease Models, Animal; D007668:Kidney / Q000000981:diagnostic imaging / Q000187:drug effects* / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D012995:Solubility / Q000187:drug effects; D008063:Thioctic Acid / Q000494:pharmacology*; D052878:Urolithiasis / Q000000981:diagnostic imaging / Q000378:metabolism*; D055114:X-Ray Microtomography
D026923:Amino Acid Transport Systems, Basic; D026921:Amino Acid Transport Systems, Neutral; C489858:Slc3a1 protein, mouse; D003553:Cystine; D008063:Thioctic Acid