A new truncating MPZ mutation associated with a very mild CMT1 B phenotype

Neuromuscul Disord. 2010 Dec;20(12):817-9. doi: 10.1016/j.nmd.2010.08.003. Epub 2010 Sep 17.

Abstract

We have investigated a 34-year-old female who had mild clinical and electrophysiological features of demyelinating peripheral neuropathy. She presented a novel frameshift mutation (V160fsX3) in the exon 4 of the Myelin Protein Zero (MPZ) gene. Clinical and genetic studies performed on her family revealed the same mutation in her oligosymptomatic mother and sister. Our report expands the number of MPZ mutations and indicates that mutations in exon 4 may cause a mild Charcot-Marie-Tooth type 1B phenotype.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Charcot-Marie-Tooth Disease / genetics
  • Female
  • Genetic Testing
  • Humans
  • Mutation
  • Myelin P0 Protein / genetics*
  • Pedigree
  • Phenotype
  • Severity of Illness Index

Substances

  • Myelin P0 Protein