Analysis of the benefits of vitamin cocktails in treating Charcot-Marie-Tooth disease type 1A

Muscle Nerve. 2008 Aug;38(2):1052-4. doi: 10.1002/mus.21071.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line, Transformed
  • Drug Combinations
  • Gene Expression Regulation / drug effects
  • Humans
  • Mice
  • Myelin Proteins / genetics
  • Myelin Proteins / metabolism*
  • Schwann Cells / drug effects
  • Schwann Cells / metabolism
  • Transfection
  • Vitamins / pharmacology*

Substances

  • Drug Combinations
  • Myelin Proteins
  • PMP22 protein, human
  • Vitamins