{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,7]],"date-time":"2026-03-07T12:18:51Z","timestamp":1772885931467,"version":"3.50.1"},"reference-count":42,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2015,11,24]],"date-time":"2015-11-24T00:00:00Z","timestamp":1448323200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["CA06927"],"award-info":[{"award-number":["CA06927"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01GM098772"],"award-info":[{"award-number":["R01GM098772"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2016,1]]},"abstract":"<jats:p>Cystathionine \u03b2 synthase (CBS) is a key enzyme in the methionine and cysteine metabolic pathway, acting as a metabolic gatekeeper to regulate the flow of fixed sulfur from methionine to cysteine. Mutations in the CBS gene cause clinical CBS deficiency, a disease characterized by elevated plasma total homocysteine (tHcy) and methionine and decreased plasma cysteine. The treatment goal for CBS\u2010deficient patients is to normalize the metabolic values of these three metabolites using a combination of vitamin therapy and dietary manipulation. To better understand the effectiveness of nutritional treatment strategies, we have performed a series of long\u2010term dietary manipulation studies using our previously developed<jats:italic>Tg\u2010I278T Cbs<jats:sup>\u2013\/\u2013<\/jats:sup><\/jats:italic>mouse model of CBS deficiency and sibling<jats:italic>Tg\u2010I278T Cbs<jats:sup>+\/\u2013<\/jats:sup><\/jats:italic>controls.<jats:italic>Tg\u2010I278T Cbs<jats:sup>\u2013\/\u2013<\/jats:sup><\/jats:italic>mice have undetectable levels of CBS activity, extremely elevated plasma tHcy, modestly elevated plasma methionine, and low plasma cysteine. They exhibit several easily assayable phenotypes, including osteoporosis, loss of fat mass, reduced life span, and facial alopecia. The diets used in these studies differed in the amounts of sulfur amino acids or sulfur amino acid precursors. In this review, we will discuss our findings and their relevance to CBS deficiency and the concept of gene\u2013diet interaction.<\/jats:p>","DOI":"10.1111\/nyas.12967","type":"journal-article","created":{"date-parts":[[2015,11,25]],"date-time":"2015-11-25T06:07:16Z","timestamp":1448431636000},"page":"80-90","source":"Crossref","is-referenced-by-count":13,"title":["The effect of dietary modulation of sulfur amino acids on cystathionine \u03b2 synthase\u2013deficient mice"],"prefix":"10.1111","volume":"1363","author":[{"given":"Warren D.","family":"Kruger","sequence":"first","affiliation":[{"name":"Cancer Biology Program Fox Chase Cancer Center Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sapna","family":"Gupta","sequence":"additional","affiliation":[{"name":"Cancer Biology Program Fox Chase Cancer Center Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,11,24]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi001088w"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.1988.255.3.E322"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)37847-5"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(1999)13:5<362::AID-HUMU4>3.0.CO;2-K"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/44.8.1833"},{"key":"e_1_2_8_7_1","first-page":"2007","volume-title":"The Metabolic Basis of Inherited Disease","author":"Mudd S.H.","year":"2001"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.09.009"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005445132327"},{"key":"e_1_2_8_10_1","first-page":"731","article-title":"Homocystinuria: a study with low\u2010methionine diet in three patients","volume":"99","author":"Sardharwalla I.B.","year":"1968","journal-title":"Can. 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