Familial occurrence of Duchenne dystrophy through paternal lines in four families

Am J Med Genet. 1991 Jan;38(1):80-4. doi: 10.1002/ajmg.1320380118.

Abstract

In a survey of 454 families with patients affected with Duchenne muscular dystrophy (DMD) we have found 4 genealogies with 2 or more affected patients who were related through paternal lines. In 1 of these families, 2 affected cousins showed different DNA deletions suggesting 2 independent mutations; in the other 2, in which only the propositus could be studied DNA deletions were also detected in the dystrophin gene. In the last one, with 3 affected patients, no DNA deletions were detected but immunohistochemical study of muscle biopsies showed a negative dystrophin pattern typical of DMD. Although one of these families might have occurred by chance, the probability of finding the other 3 in our sample of families with DMD patients constitutes a rare event. It is suggested that other mechanisms, such as the presence of transposable elements in other sites of the genome, could be responsible in some families, for a greater predisposition for the occurrence of pathogenic deletions, duplications or mutations.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Chromosome Deletion*
  • DNA / analysis*
  • DNA Transposable Elements
  • Dystrophin / genetics
  • Female
  • Genes, Recessive
  • Humans
  • Male
  • Mosaicism
  • Muscular Dystrophies / genetics*
  • Pedigree
  • X Chromosome / ultrastructure*

Substances

  • DNA Transposable Elements
  • Dystrophin
  • DNA