{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T09:17:45Z","timestamp":1789204665071,"version":"build-2803163510"},"reference-count":31,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,11,4]],"date-time":"2013-11-04T00:00:00Z","timestamp":1383523200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100004897","name":"Pennsylvania Department of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["CA06927"],"award-info":[{"award-number":["CA06927"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01GM098772"],"award-info":[{"award-number":["R01GM098772"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:p>\n                    Cystathionine \u03b2\u2010synthase (CBS) deficiency is a recessive inborn error of metabolism characterized by elevated serum total homocysteine (tHcy). Previously, our laboratory developed a mouse model of CBS deficiency,\n                    <jats:italic>\n                      TgI278T Cbs\n                      <jats:sup>\u2010<\/jats:sup>\n                      \/\n                      <jats:sup>\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    (abbreviated as\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                      ),\n                    <\/jats:italic>\n                    characterized by low weight, low adiposity, decreased\n                    <jats:italic>Scd\u20101<\/jats:italic>\n                    expression, facial alopecia, and osteoporosis. To determine the potential benefit of a methionine\u2010restricted diet (MRD), we fed\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    and\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>+\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    control mice either an MRD or a regular diet (RD) from weaning till 240 d of age.\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice fed the MRD had a 77% decrease in tHcy, 28% increase in weight, 130% increase in fat mass, 82% increase in\n                    <jats:italic>Scd\u20101<\/jats:italic>\n                    expression, and 10.6% increase in bone density and entirely lacked the alopecia phenotype observed in age\u2010matched\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice fed the RD. At the end of the study,\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice fed the MRD were phenotypically indistinguishable from\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>+\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice fed the RD. Notably, whereas the MRD diet was highly beneficial to\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice, it had nearly opposite effect on\n                    <jats:italic>\n                      Cbs\n                      <jats:sup>+\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice. These studies show that a low\u2010methionine diet can correct the phenotypic consequences of loss of CBS and provide a striking example of how genotype and diet can interact to influence phenotype in mammals.\u2014Gupta, S., Melnyk, S.B., Kruger, W.D. Cystathionine \u03b2\u2010synthase\u2010deficient mice thrive on a low\u2010methionine diet.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    28, 781\u2013790 (2014).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.13-240770","type":"journal-article","created":{"date-parts":[[2013,11,4]],"date-time":"2013-11-04T20:57:32Z","timestamp":1383598652000},"page":"781-790","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Cystathionine \u03b2\u2010synthase\u2010deficient mice thrive on a low\u2010methionine diet"],"prefix":"10.1096","volume":"28","author":[{"given":"Sapna","family":"Gupta","sequence":"first","affiliation":[{"name":"Cancer Biology Program Fox Chase Cancer Center Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stepan B.","family":"Melnyk","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Arkansas for Medical Sciences Little Rock Arkansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warren D.","family":"Kruger","sequence":"additional","affiliation":[{"name":"Cancer Biology Program Fox Chase Cancer Center Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,11,4]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"1","article-title":"The natural history of homocystinuria due to cystathionine beta\u2010synthase deficiency","volume":"37","author":"Mudd S. 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