Replication errors may contribute to the generation of large deletions and duplications in the dystrophin gene

Hum Mutat. 1992;1(4):280-7. doi: 10.1002/humu.1380010403.

Abstract

Frequent recurrent mutations of the human dystrophin gene lead to Duchenne and Becker muscular dystrophies. Although the approximately 2.5 Mb size of the gene may form a large target for mutations it is not clear to date which mechanisms promote the observed high frequency of spontaneous mutants (1 in 10,000 X-chromosomes) of which a high percentage (> 70%) are gross structural aberrations (deletions/duplications). In order to gain insight into possible molecular mechanisms we have cloned and sequenced the deletion junction fragments from two unrelated Duchenne patients. Our data, together with a short review on other cases from the literature, present evidence that errors of DNA replication may contribute to the generation of submicroscopic chromosome rearrangements.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Cloning, Molecular
  • DNA / genetics
  • DNA Mutational Analysis
  • DNA Replication*
  • Dystrophin / genetics*
  • Gene Rearrangement
  • Humans
  • Male
  • Molecular Sequence Data
  • Multigene Family
  • Muscular Dystrophies / genetics
  • Restriction Mapping
  • Sequence Deletion

Substances

  • Dystrophin
  • DNA