Fragile X full mutations are more similar in siblings than in unrelated patients: further evidence for a familial factor in CGG repeat dynamics

Genet Med. 2000 Jul-Aug;2(4):242-8. doi: 10.1097/00125817-200007000-00007.

Abstract

Purpose: We sought to compare patterns of full mutation repeat-length variability in the peripheral blood DNA of patients with fragile X syndrome to determine whether siblings possess mutation patterns more similar than those of unrelated patients.

Methods: Mutation patterns were visualized by Southern blot analysis and captured digitally with a phosphor imager. Novel comparison strategies based on overlapping profile plots and calculation of weighted mean CGG repeat values were used to assess mutation pattern similarity.

Results: Within the population that we analyzed of 56 patients with full mutation, mutation patterns were found to be more similar in siblings than in unrelated patients.

Conclusion: These results indicate that repeat-length variability may be generated in a nonrandom manner and that familial factors influence this process.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Blotting, Southern
  • Family Health
  • Fragile X Syndrome / genetics*
  • Genetic Variation
  • Humans
  • Models, Statistical
  • Mutation*
  • Nuclear Family
  • Pedigree
  • Trinucleotide Repeats