title: Analysis of the benefits of vitamin cocktails in treating Charcot-Marie-Tooth disease type 1A.
abstract: We recently proposed that the use of high doses of ascorbic acid (AA) could constitute the first potential treatment for Charcot-Marie-Tooth disease type 1A (CMT1A).4 We investigated the potential benefits of using cocktails of vitamins for CMT1A therapy. We used transient transfection of Schwann cells with a construction placing the expression of a reporter gene under the control of the Schwann cell-specific promoter of PMP22. Transfected cells were cultured with or without addition of ascorbic acid, vitamin A, vitamin E, or a cocktail of these vitamins. Adding vitamin A or E counteracts the effect of ascorbic acid in inhibiting PMP22 expression. We thus recommend that vitamins A and E should not be included in combination with AA in clinical trials.
authors.affiliation: INSERM UMR491, TMG, IPHM-IFR125, Faculté de Médecine, 27 bd Jean Moulin, 13385 Marseille Cedex 5, France.
authors.forename: Ferdinand
authors.initials: F
authors.lastname: Kaya
authors.forename: Sophie
authors.initials: S
authors.lastname: Belin
authors.forename: Joelle
authors.initials: J
authors.lastname: Micallef
authors.forename: Olivier
authors.initials: O
authors.lastname: Blin
authors.forename: Michel
authors.initials: M
authors.lastname: Fontés
mesh_terms: D000818:Animals; D002461:Cell Line, Transformed; D004338:Drug Combinations; D005786:Gene Expression Regulation / Q000187:drug effects; D006801:Humans; D051379:Mice; D009185:Myelin Proteins / Q000235:genetics / Q000378:metabolism*; D012583:Schwann Cells / Q000187:drug effects / Q000378:metabolism; D014162:Transfection; D014815:Vitamins / Q000494:pharmacology*
chemical_list: D004338:Drug Combinations; D009185:Myelin Proteins; C073687:PMP22 protein, human; D014815:Vitamins