{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T21:15:22Z","timestamp":1791494122729,"version":"4.3.4"},"reference-count":49,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2017,12,19]],"date-time":"2017-12-19T00:00:00Z","timestamp":1513641600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2017,12,19]],"date-time":"2017-12-19T00:00:00Z","timestamp":1513641600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:p>\n                    Cystathionine \u03b2\u2010synthase (CBS) catalyzes the transsulfuration pathway and contributes, among other functions, to the generation of hydrogen sulfide. In view of the exceptionally high expression of CBS in the liver and the common interleukin\u20106 pathway used in the regulatory systems of hydrogen sulfide and hepcidin, we speculate that CBS is involved in body iron homeostasis. We found that CBS knockout (CBS\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    ) mice exhibited anemia and a significant increase in iron content in the serum, liver, spleen, and heart, along with severe damage to the liver, displaying a hemochromatosis\u2010like phenotype. A high level of hepatic and serum hepcidin was also found. A major cause of the systemic iron overload is the reduced iron usage due to suppressed erythropoiesis, which is consistent with an increase in interleukin\u20106 and reduced expression of erythropoietin. Importantly, in the liver, absence of CBS caused both a reduction in the transcriptional factor nuclear factor erythroid 2\u2013related factor\u20102 and an up\u2010regulation of hepcidin that led to a decrease in the iron export protein ferroportin 1. The resulting suppression of iron export exacerbates iron retention, causing damage to hepatocytes. Finally, administration of CBS\u2010overexpressing adenovirus into CBS mutant mice could partially reverse the iron\u2010related phenotype.\n                    <jats:italic toggle=\"yes\">Conclusion<\/jats:italic>\n                    : Our findings point to a critical role of CBS in iron homeostasis of the body, and the liver in particular; it is likely that a hemochromatosis\u2010like phenotype in patients can be induced by aberration not only in the expression of key molecules in the hepcidin pathway but also of those related to CBS. (H\n                    <jats:sc>epatology<\/jats:sc>\n                    2018;67:21\u201035)\n                  <\/jats:p>","DOI":"10.1002\/hep.29499","type":"journal-article","created":{"date-parts":[[2017,8,31]],"date-time":"2017-08-31T12:52:36Z","timestamp":1504183956000},"page":"21-35","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":40,"title":["Cystathionine \u03b2\u2010synthase is required for body iron homeostasis"],"prefix":"10.1097","volume":"67","author":[{"given":"Yu\u2010Fu","family":"Zhou","sequence":"first","affiliation":[{"name":"Laboratory of Neuropharmacology,Fudan University School of Pharmacy,Shanghai,China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao\u2010Mei","family":"Wu","sequence":"additional","affiliation":[{"name":"School of Biomedical Sciences, Faculty of Medicine,The Chinese University of Hong Kong,Shatin,NT,Hong 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