Identification of a novel missense mutation in Wilson's disease gene

Chin Med J (Engl). 1997 Nov;110(11):887-90.

Abstract

Objective: To investigate the allelic heterogeneity of the ATP7B gene in Chinese patients with Wilson's disease (WD).

Methods: Exons of the ATP7B gene from 141 WD patients' DNA were amplified with polymerase chain reaction (PCR) 887-890. Mutations were then screened by single strand conformation polymorphism (SSCP) analysis and further identified by sequencing.

Results: The molecular structure of exon 7 of the ATP7B gene from 141 WD patients was analyzed. The same band shift in electrophoretic pattern of 4 cerebral type patients was identified with SSCP and subsequently sequenced. The results showed missense mutation at the second base of the codon as Ser 662 Cys, which is caused by a C to G transversion.

Conclusions: Mutations of the ATP7B gene were investigated for the first time in China and a novel missense mutation was identified in four cases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Base Sequence
  • Chromosomes, Human, Pair 13
  • Female
  • Hepatolenticular Degeneration / genetics*
  • Humans
  • Male
  • Molecular Sequence Data
  • Mutation, Missense*
  • Phenotype
  • Polymerase Chain Reaction
  • Polymorphism, Single-Stranded Conformational
  • Sequence Analysis, DNA