Dystrophin Exon 29 Nonsense Mutations Cause a Variably Mild Phenotype

Ulster Med J. 2017 Sep;86(3):185-188. Epub 2017 Sep 12.

Abstract

Background: Nonsense mutations in the dystrophin gene usually result in a severe Duchenne muscular dystrophy phenotype.

Findings: We describe a 7-year-old boy with a rare pathogenic mutation in exon 29 c.3940C>T p.(Arg1314Ter) resulting in exon skipping, in turn rescuing the phenotype from a severe Duchenne type to a milder Becker muscular dystrophy type. No adults have been described with this mutation to date.

Conclusions: Exon skipping of exon 29 results in a higher level of functional dystrophin. Some cases of muscular dystrophy may still require muscle biopsy to determine optimal management and pharmaceutical treatment options.

Keywords: Becker muscular dystrophy; Dystrophin; Exon 29; Exon skipping; Nonsense mutation.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Codon, Nonsense
  • Dystrophin / genetics*
  • Exons
  • Humans
  • Male
  • Muscular Dystrophy, Duchenne / genetics*
  • Phenotype
  • Severity of Illness Index

Substances

  • Codon, Nonsense
  • Dystrophin