Thioethers as markers of hydrogen sulfide production in homocystinurias

Biochimie
V KozichJ P Kraus

Abstract

Two enzymes in the transsulfuration pathway of homocysteine -cystathionine beta-synthase (CBS) and gamma-cystathionase (CTH)-use cysteine and/or homocysteine to produce the important signaling molecule hydrogen sulfide (H2S) and simultaneously the thioethers lanthionine, cystathionine or homolanthionine. In this study we explored whether impaired flux of substrates for H2S synthesis and/or deficient enzyme activities alter production of hydrogen sulfide in patients with homocystinurias. As an indirect measure of H2S synthesis we determined by LC-MS/MS concentrations of thioethers in plasma samples from 33 patients with different types of homocystinurias, in 8 patient derived fibroblast cell lines, and as reaction products of seven purified mutant CBS enzymes. Since chaperoned recombinant mutant CBS enzymes retained capacity of H2S synthesis in vitro it can be stipulated that deficient CBS activity in vivo may impair H2S production. Indeed, in patients with classical homocystinuria we observed significantly decreased cystathionine and lanthionine concentrations in plasma (46% and 74% of median control levels, respectively) and significantly lower cystathionine in fibroblasts (8% of median control concentrations) indicating that ...Continue Reading

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Citations

Oct 25, 2016·International Journal of Molecular Sciences·Henrieta ŠkovierováJán Lehotský
Sep 7, 2017·Antioxidants & Redox Signaling·Tomas MajtanJan P Kraus
Oct 21, 2018·British Journal of Pharmacology·Viktor KožichPeter Nagy
Nov 14, 2018·Canadian Journal of Physiology and Pharmacology·Avisek MajumderSuresh C Tyagi
Aug 20, 2017·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Tomas MajtanJan P Kraus
Jul 28, 2018·Oxidative Medicine and Cellular Longevity·Alessandro Giuffrè, João B Vicente
Oct 1, 2020·Molecular Medicine·Maurizio Dattilo
Oct 2, 2020·The Journal of Nutrition·Viktor Kožich, Sally Stabler
Oct 17, 2020·Oxidative Medicine and Cellular Longevity·Antonio FranciosoMario Fontana

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