Thioethers as markers of hydrogen sulfide production in homocystinurias
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- 作者：Viktor Kožicha ; viktor.kozich@vfn.cz" class="auth_mail" title="E-mail the corresponding author ; Jakub Krijta ; Jitka Sokolová ; a ; Petra Melenovská ; a ; Pavel Je&scaron ; inaa ; Roman Vozdeka ; 1 ; Tomá ; &scaron ; Majtá ; nb ; Jan P. Krausb
- 关键词：Hydrogen sulfide ; Cystathionine beta-synthase ; Gamma-cystathionase ; Homolanthionine ; Cystathionine ; Lanthionine
- 刊名：Biochimie
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：126
- 期：Complete
- 页码：14-20
- 全文大小：905 K
文摘
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Seven different CBS mutants can produce thioethers from cysteine and homocysteine.
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Metabolism of thioethers is deranged in disorders of sulfur amino acid metabolism.
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Cystathionine and lanthionine are decreased in plasma of CBS deficient patients.
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In homocystinurias homolanthionine produced by CTH correlates with total homocysteine.
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All these data suggest altered hydrogen sulfide metabolism in homocystinurias.