Two families with Xq27.3 fragility, no detectable insert in the FMR-1 gene, mild mental impairment, and absence of the Martin-Bell phenotype

Am J Med Genet. 1992;43(1-2):232-6. doi: 10.1002/ajmg.1320430137.

Abstract

In 2 families, propositi were investigated because of mild developmental delay and, in one case, behavior disorders. Seven males in the 2 families were found to have a fragile site at Xq27.3 but the usual insert in the FMR-1 gene was absent. The affected males had mild, or in some cases, no clear intellectual impairment and did not have the Martin-Bell phenotype. Carrier females in one family tended to show a high level of cytogenetic expression of the fragile site but were clinically normal. It is not yet clear whether these families have unusual mutations in the FMR-1 gene or whether their fragile sites are different, but cytogenetically indistinguishable from, that associated with inserts in the FMR-1 gene.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Child, Preschool
  • Chromosome Fragile Sites
  • Chromosome Fragility*
  • DNA Mutational Analysis
  • Female
  • Fragile X Syndrome / genetics*
  • Fragile X Syndrome / pathology
  • Fragile X Syndrome / psychology
  • Heterozygote
  • Humans
  • Intelligence
  • Male
  • Pedigree
  • Phenotype
  • X Chromosome*