{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T01:07:56Z","timestamp":1792112876433,"version":"4.4.0"},"reference-count":123,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,1,28]],"date-time":"2019-01-28T00:00:00Z","timestamp":1548633600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001711","name":"Schweizerischer Nationalfonds zur F\u00f6rderung der Wissenschaftlichen Forschung","doi-asserted-by":"publisher","award":["SNSF 31003A_156907"],"award-info":[{"award-number":["SNSF 31003A_156907"]}],"id":[{"id":"10.13039\/501100001711","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001711","name":"Schweizerischer Nationalfonds zur F\u00f6rderung der Wissenschaftlichen Forschung","doi-asserted-by":"publisher","award":["SNSF 31003A_175779"],"award-info":[{"award-number":["SNSF 31003A_175779"]}],"id":[{"id":"10.13039\/501100001711","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006447","name":"Universit\u00e4t Z\u00fcrich","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006447","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Vitamin B\n                    <jats:sub>12<\/jats:sub>\n                    (cobalamin, Cbl) is a nutrient essential to human health. Due to its complex structure and dual cofactor forms, Cbl undergoes a complicated series of absorptive and processing steps before serving as cofactor for the enzymes methylmalonyl\u2010CoA mutase and methionine synthase. Methylmalonyl\u2010CoA mutase is required for the catabolism of certain (branched\u2010chain) amino acids into an anaplerotic substrate in the mitochondrion, and dysfunction of the enzyme itself or in production of its cofactor adenosyl\u2010Cbl result in an inability to successfully undergo protein catabolism with concomitant mitochondrial energy disruption. Methionine synthase catalyzes the methyl\u2010Cbl dependent (re)methylation of homocysteine to methionine within the methionine cycle; a reaction required to produce this essential amino acid and generate S\u2010adenosylmethionine, the most important cellular methyl\u2010donor. Disruption of methionine synthase has wide\u2010ranging implications for all methylation\u2010dependent reactions, including epigenetic modification, but also for the intracellular folate pathway, since methionine synthase uses 5\u2010methyltetrahydrofolate as a one\u2010carbon donor. Folate\u2010bound one\u2010carbon units are also required for deoxythymidine monophosphate and de novo purine synthesis; therefore, the flow of single carbon units to each of these pathways must be regulated based on cellular needs. This review provides an overview on Cbl metabolism with a brief description of absorption and intracellular metabolic pathways. It also provides a description of folate\u2010mediated one\u2010carbon metabolism and its intersection with Cbl at the methionine cycle. Finally, a summary of recent advances in understanding of how both pathways are regulated is presented.\n                  <\/jats:p>","DOI":"10.1002\/jimd.12009","type":"journal-article","created":{"date-parts":[[2019,1,28]],"date-time":"2019-01-28T23:59:59Z","timestamp":1548719999000},"page":"673-685","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":470,"title":["Vitamin B\n                    <sub>12<\/sub>\n                    , folate, and the methionine remethylation cycle\u2014biochemistry, pathways, and regulation"],"prefix":"10.1002","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1557-3517","authenticated-orcid":false,"given":"D. 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