Fragile X mutation and FG syndrome-like phenotype

Am J Med Genet. 1996 Aug 9;64(2):395-8. doi: 10.1002/(SICI)1096-8628(19960809)64:2<395::AID-AJMG32>3.0.CO;2-8.

Abstract

We present data on 4 mentally retarded brothers, 2 of whom were dizygotic twins with congenital hypotonia, constipation, head size disproportionately large for length or height, and a combination of minor anomalies suggestive of FG syndrome. These brothers have a mentally retarded full sister with similar minor anomalies and an older half-brother with the Martin-Bell syndrome. The mother is mentally retarded; 4 of 7 individuals are positive for fragile X, but all have a CGG expansion ranging from 0.2-2 to 4 kb. Although the phenotype is not completely typical of the FG syndrome and the coincidence of the FMR1 mutation and segregation of the MCA/MR phenotype are highly unlikely, the FMR1 mutation may affect morphogenesis more extensively and differently than the Martin-Bell syndrome does to effect an FG syndromelike phenotype in certain families. This phenotype does not appear to be a contiguous gene syndrome, but an effect of the FMR1 mutation on an adjacent gene must be considered.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Diseases in Twins
  • Female
  • Fragile X Mental Retardation Protein
  • Fragile X Syndrome / genetics*
  • Humans
  • Infant
  • Intellectual Disability / genetics
  • Male
  • Morphogenesis
  • Nerve Tissue Proteins / genetics
  • Pedigree
  • Phenotype
  • RNA-Binding Proteins*
  • Sex Chromosome Aberrations / genetics*
  • Syndrome
  • Trinucleotide Repeats

Substances

  • FMR1 protein, human
  • Nerve Tissue Proteins
  • RNA-Binding Proteins
  • Fragile X Mental Retardation Protein