[X-linked recessive Charcot-Marie-Tooth disease and Cx32 gene mutation]

Zhonghua Nei Ke Za Zhi. 2001 Aug;40(8):543-5.
[Article in Chinese]

Abstract

Objective: To study the Cx32 gene mutation in a X-linked recessive Charcot-Marie-Tooth disease (CMTXR) family.

Methods: Mutation analysis of Cx32 was screened by polymerase chain reaction single strand conformation polymorphism (PCR-SSCP) combined with DNA direct sequencing in 4 patients, 9 unaffected family members and 50 unrelated normal individuals.

Results: Arg15Gln mutation was found in 4 patients and 3 unaffected family members.

Conclusion: The mutation of Cx32 can also cause CMTXR. Using PCR-SSCP combined with DNA direct sequencing can make gene diagnosis to CMTXR that is caused by Cx32 mutation.

Publication types

  • English Abstract

MeSH terms

  • Adult
  • Amino Acid Substitution
  • Charcot-Marie-Tooth Disease / genetics*
  • Connexins / genetics*
  • Female
  • Gap Junction beta-1 Protein
  • Humans
  • Male
  • Middle Aged
  • Mutation
  • Pedigree
  • X Chromosome*

Substances

  • Connexins