{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T06:26:24Z","timestamp":1785392784622,"version":"3.55.0"},"reference-count":65,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2015,5,12]],"date-time":"2015-05-12T00:00:00Z","timestamp":1431388800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Molecular Nutrition Food Res"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:p>Methylcobalamin (MeCbl) and adenosylcobalamin (AdoCbl) are coenzymes for methionine synthase and methylmalonyl\u2010CoA mutase, respectively. Hydroxylcobalamin (HOCbl) and cyanocobalamin (CNCbl) are frequently used for supplementation. MeCbl and AdoCbl have recently emerged as alternative forms in supplements. In the light of metabolic transformation of Cbl into its cofactor forms, this review discusses current evidence on efficacy and utility of different Cbl forms in preventing or treating Cbl deficiency. Cbl\u2010transporting proteins bind and mediate the uptake of all aforementioned forms of Cbl. After internalization and lysosomal release, Cbl binds to the cytosolic chaperon MMACHC that is responsible for (i) flavin\u2010dependent decyanation of [CN\u2010Co<jats:sup>3+<\/jats:sup>]Cbl to [Co<jats:sup>2+<\/jats:sup>]Cbl; (ii) glutathione\u2010dependent dealkylation of MeCbl and AdoCbl to [Co<jats:sup>2+\/1+<\/jats:sup>]Cbl; and (iii) glutathione\u2010dependent decyanation of CNCbl or reduction of HOCbl under anaerobic conditions. MMACHC shows a broad specificity for Cbl forms and supplies the Cbl<jats:sup>2+<\/jats:sup> intermediate for synthesis of MeCbl and AdoCbl. Cobalamin chemistry, physiology, and biochemistry suggest that MeCbl and AdoCbl follow the same route of intracellular processing as CNCbl does. We conclude that supplementing MeCbl or AdoCbl is unlikely to be advantageous compared to CNCbl. On the other hand, there are obvious advantages of high parenteral doses (1\u20132 mg) of HOCbl in treating inborn errors of Cbl metabolism.<\/jats:p>","DOI":"10.1002\/mnfr.201500019","type":"journal-article","created":{"date-parts":[[2015,3,29]],"date-time":"2015-03-29T06:44:26Z","timestamp":1427611466000},"page":"1364-1372","source":"Crossref","is-referenced-by-count":103,"title":["Cobalamin coenzyme forms are not likely to be superior to cyano\u2010 and hydroxyl\u2010cobalamin in prevention or treatment of cobalamin deficiency"],"prefix":"10.1002","volume":"59","author":[{"given":"Rima","family":"Obeid","sequence":"first","affiliation":[{"name":"Aarhus Institute of Advanced Studies,  University of Aarhus  Aarhus Denmark"},{"name":"Department of Clinical Chemistry University Hospital of the Saarland  Homburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergey N.","family":"Fedosov","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry Aarhus University Hospital  Aarhus C Denmark"},{"name":"Department of Molecular Biology and Genetics Aarhus University  Aarhus C Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ebba","family":"Nexo","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry Aarhus University Hospital  Aarhus C Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,5,12]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/178064a0"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/192937a0"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejcn.2014.165"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1517\/13543784.17.6.953"},{"key":"e_1_2_9_6_1","first-page":"306","volume-title":"Vitamin B12. 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