Mutations in the Myelin Protein Zero result in a spectrum of Charcot-Marie-Tooth phenotypes

Acta Myol. 2004 May;23(1):6-9.

Abstract

Initially the Myelin Protein Zero gene was shown to be mutated in the demyelinating form of Charcot-Marie-Tooth disease (CMT1). The vast majority of the mutations in the Myelin Protein Zero gene have been detected in the Charcot-Marie-Tooth (1B) disease, however, some of them were found in patients suffering from congenital hypomyelinating neuropathy and axonal type Charcot-Marie-Tooth disease. In this study, a Charcot-Marie-Tooth disease phenotype diversity associated with different mutations in the MPZ gene, is described.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Charcot-Marie-Tooth Disease / genetics*
  • Humans
  • Mutation
  • Myelin P0 Protein / genetics*
  • Phenotype

Substances

  • Myelin P0 Protein