Three novel mutations of the NF1 gene detected by temperature gradient gel electrophoresis of exons 5 and 8

Electrophoresis. 1996 Oct;17(10):1559-63. doi: 10.1002/elps.1150171011.

Abstract

We screened a total of 100 unrelated patients with neurofibromatosis type 1 (NF1) for mutations on exons 5 and 8 of the NF1 gene using temperature gradient gel electrophoresis (TGGE). Careful interpretation of exon 5 TGGE patterns was necessary due to interference by an exonic polymorphism. Three novel mutations were identified: a stop mutation in exon 5 (Q239X) caused by a C-->T transition at cDNA nucleotide position 715, a transition at the invariant G of the splice acceptor site in the intron 4c (G655-1A), and a transversion at the invariant G of the splice donor site in intron 8 (G1185 + 1T). Analysis of mRNA revealed the predicted abnormal splice products. While skipping of exon 5 causes a shift in the reading frame with a premature stop codon downstream in the middle of exon 6, skipping of exon 8 leads to an in-frame deletion with the predicted protein product being shortened by 41 amino acids.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Electrophoresis, Polyacrylamide Gel*
  • Exons*
  • Genes, Neurofibromatosis 1 / genetics*
  • Humans
  • Molecular Sequence Data
  • Mutation*
  • Pedigree
  • Polymerase Chain Reaction
  • Polymorphism, Genetic
  • RNA Splicing
  • RNA, Messenger / analysis
  • Sequence Analysis, DNA
  • Temperature

Substances

  • RNA, Messenger

Associated data

  • GENBANK/U17662
  • GENBANK/U17667