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Article

S-Methyl-L-Ergothioneine to L-Ergothioneine Ratio in Urine Is a Marker of Cystine Lithiasis in a Cystinuria Mouse Model

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Human Molecular Genetics Laboratory, Gene, Disease and Therapy Program, IDIBELL, L’Hospitalet de Llobregat, 08908 Barcelona, Spain
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Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER)-CB06/07/0069, Instituto de Salud Carlos III, 28029 Madrid, Spain
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SIMChem, Institute for Advanced Chemistry of Catalonia (IQAC-CSIC), 08034 Barcelona, Spain
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Department of Biomedical Sciences, University of Sassari, 07100 Sassari, Italy
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MCS, Laboratory of Medicinal Chemistry, Institute for Advanced Chemistry of Catalonia (IQAC-CSIC), 08034 Barcelona, Spain
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Faculty of Pharmacy, Kanazawa University, Kanazawa 920-1192, Japan
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Genetics Section, Physiological Sciences Department, Health Sciences and Medicine Faculty, University of Barcelona, 08907 Barcelona, Spain
*
Authors to whom correspondence should be addressed.
Academic Editor: Holger Husi
Antioxidants 2021, 10(9), 1424; https://doi.org/10.3390/antiox10091424
Received: 28 July 2021 / Revised: 30 August 2021 / Accepted: 31 August 2021 / Published: 7 September 2021
Cystinuria, a rare inherited aminoaciduria condition, is characterized by the hyperexcretion of cystine, ornithine, lysine, and arginine. Its main clinical manifestation is cystine stone formation in the urinary tract, being responsible for 1–2% total and 6–8% pediatric lithiasis. Cystinuria patients suffer from recurrent lithiasic episodes that might end in surgical interventions, progressive renal functional deterioration, and kidney loss. Cystinuria is monitored for the presence of urinary cystine stones by crystalluria, imaging techniques or urinary cystine capacity; all with limited predicting capabilities. We analyzed blood and urine levels of the natural antioxidant L-ergothioneine in a Type B cystinuria mouse model, and urine levels of its metabolic product S-methyl-L-ergothioneine, in both male and female mice at two different ages and with different lithiasic phenotype. Urinary levels of S-methyl-L-ergothioneine showed differences related to age, gender and lithiasic phenotype. Once normalized by L-ergothioneine to account for interindividual differences, the S-methyl-L-ergothioneine to L-ergothioneine urinary ratio discriminated between cystine lithiasic phenotypes. Urine S-methyl-L-ergothioneine to L-ergothioneine ratio could be easily determined in urine and, as being capable of discriminating between cystine lithiasis phenotypes, it could be used as a lithiasis biomarker in cystinuria patient management. View Full-Text
Keywords: L-ergothioneine; S-methyl-L-ergothioneine; cystine lithiasis biomarker; cystinuria; Slc7a9 KO mice L-ergothioneine; S-methyl-L-ergothioneine; cystine lithiasis biomarker; cystinuria; Slc7a9 KO mice
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MDPI and ACS Style

López de Heredia, M.; Muñoz, L.; Carru, C.; Sotgia, S.; Zinellu, A.; Serra, C.; Llebaria, A.; Kato, Y.; Nunes, V. S-Methyl-L-Ergothioneine to L-Ergothioneine Ratio in Urine Is a Marker of Cystine Lithiasis in a Cystinuria Mouse Model. Antioxidants 2021, 10, 1424. https://doi.org/10.3390/antiox10091424

AMA Style

López de Heredia M, Muñoz L, Carru C, Sotgia S, Zinellu A, Serra C, Llebaria A, Kato Y, Nunes V. S-Methyl-L-Ergothioneine to L-Ergothioneine Ratio in Urine Is a Marker of Cystine Lithiasis in a Cystinuria Mouse Model. Antioxidants. 2021; 10(9):1424. https://doi.org/10.3390/antiox10091424

Chicago/Turabian Style

López de Heredia, Miguel, Lourdes Muñoz, Ciriaco Carru, Salvatore Sotgia, Angelo Zinellu, Carmen Serra, Amadeu Llebaria, Yukio Kato, and Virginia Nunes. 2021. "S-Methyl-L-Ergothioneine to L-Ergothioneine Ratio in Urine Is a Marker of Cystine Lithiasis in a Cystinuria Mouse Model" Antioxidants 10, no. 9: 1424. https://doi.org/10.3390/antiox10091424

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