CMT 1A: Analysis of the benefits of vitamin cocktails in treating Charcot Marie

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Muscle Nerve. 2008 Jul 28;38(2):1052-1054.

Analysis of the benefits of vitamin cocktails in treating Charcot-

Marie-Tooth disease type 1A.

Kaya F, Belin S, Micallef J, Blin O, Fontés M.

INSERM UMR491, TMG, IPHM-IFR125, Faculté de Médecine, 27 bd Jean

Moulin, 13385 Marseille Cedex 5, France.

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.