A splicing mutation in intron 16 of the cystic fibrosis transmembrane conductance regulator gene, associated with severe disease, is common on Reunion Island

Hum Hered. 1996 May-Jun;46(3):168-71. doi: 10.1159/000154347.

Abstract

In addition to the frequent delta F508 and Y122X mutations on cystic fibrosis (CF) chromosomes of patients from Reunion Island, one splicing mutation, 3120+1G-->A is observed relatively frequently (12.5%) in this group, in comparison with the French metropolitan population (<0.001 %). This mutation, disrupting the 5' splice donor site of intron 16, can be detected easily by restriction enzyme BstNI digestion. In 2 CF patients homozygous for this mutation, the clinical pattern was severe, with both pancreatic insufficiency and respiratory symptoms.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Cystic Fibrosis / epidemiology
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Humans
  • Introns*
  • Mutation
  • RNA Splicing*
  • Reunion / epidemiology

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator