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Although dietary vitamin intake is sufficient to sustain coenzyme synthesis in healthy individuals, it is insufficient in vitamin\u2010responsive IMDs, where pharmacological supplementation can restore deficient metabolic fluxes or stabilise impaired enzymes. This review provides an integrated overview of the biochemical pathways that convert vitamins into their active coenzymes and documents all currently known hereditary disorders responsive to vitamin or coenzyme therapy, along with recommended doses. For each vitamin group (B\n                    <jats:sub>1<\/jats:sub>\n                    , B\n                    <jats:sub>2<\/jats:sub>\n                    , B\n                    <jats:sub>3<\/jats:sub>\n                    , B\n                    <jats:sub>6<\/jats:sub>\n                    , B\n                    <jats:sub>8<\/jats:sub>\n                    , B\n                    <jats:sub>9<\/jats:sub>\n                    , and B\n                    <jats:sub>12<\/jats:sub>\n                    ) and BH\n                    <jats:sub>4<\/jats:sub>\n                    , we outline absorption, intracellular trafficking, coenzyme formation and turnover, major clinical phenotypes, diagnostic biomarkers, and vitamin therapeutic considerations, including dose ranges, formulation constraints, and safety issues. The expected therapeutic benefit is graded to assist with clinical decision\u2010making. As many conditions are rare and have only recently been described, the evidence is sometimes limited; therefore, systematic reporting of individual responses, including vitamin forms and dosing, remains essential. Early recognition of vitamin\u2010responsive IMDs is critical, as timely treatment can dramatically alter disease trajectories and, in some cases, fully reverse symptoms.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70207","type":"journal-article","created":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:08:09Z","timestamp":1780470489000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Vitamin\u2010Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy"],"prefix":"10.1002","volume":"49","author":[{"given":"C\u00e9cile","family":"Acquaviva","sequence":"first","affiliation":[{"name":"Inborn Error of Metabolism Unit Biochemistry and Molecular Biology Department  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1466-6957","authenticated-orcid":false,"given":"Yann","family":"Nadjar","sequence":"additional","affiliation":[{"name":"Reference Center for Metabolic and Lysosomal Neurological Diseases, Neuro\u2010Metabolism Unit Neurology Department  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8272-232X","authenticated-orcid":false,"given":"Manuel","family":"Schiff","sequence":"additional","affiliation":[{"name":"Reference Center for Inborn Errors of Metabolism, Department of Pediatrics, Necker Hospital, APHP Universit\u00e9 Paris\u2010Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8477-3120","authenticated-orcid":false,"given":"Christine","family":"Vianey\u2010Saban","sequence":"additional","affiliation":[{"name":"Inborn Error of Metabolism Unit Biochemistry and Molecular Biology Department  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Fouilhoux","sequence":"additional","affiliation":[{"name":"Lyon Reference Center for Inborn Errors of Metabolism, Department of Pediatrics Groupement Hospitalier Est  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5389-8585","authenticated-orcid":false,"given":"Jean\u2010Fran\u00e7ois","family":"Benoist","sequence":"additional","affiliation":[{"name":"Metabolic Biochemistry, Necker Hospital, APHP  Paris France"},{"name":"Paris\u2010Saclay University Faculty of Pharmacy  Orsay France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,3]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12677"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12873\u2010023\u201000851\u20105"},{"key":"e_1_2_8_4_1","unstructured":"J. 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