Pfeiffer's syndrome resulting from an S351C mutation in the fibroblast growth factor receptor-2 gene

J Craniofac Surg. 1998 May;9(3):207-9. doi: 10.1097/00001665-199805000-00004.

Abstract

For four of the most well-known craniosynostosis syndromes--Apert's, Crouzon's, Pfeiffer's, and Jackson-Weiss' syndromes--mutations in the fibroblast growth factor receptors (FGFRs) have been described. These substitutions arise mainly in the FGFR-2 gene and to a much lesser degree in the FGFR-1 and FGFR-3 genes. We present a patient with an apparently sporadic type of Pfeiffer's syndrome, exhibiting nearly all associated features of this syndrome. A mutation in the FGFR-2 gene was found, namely serine351-cysteine. This mutation has been reported in only one patient so far, whose phenotype could match both Crouzon's and Pfeiffer's syndromes.

Publication types

  • Case Reports

MeSH terms

  • Acrocephalosyndactylia / genetics*
  • Amino Acid Substitution
  • Cysteine / genetics
  • Female
  • Humans
  • Infant, Newborn
  • Point Mutation
  • Polymerase Chain Reaction
  • Receptor Protein-Tyrosine Kinases / genetics*
  • Receptor, Fibroblast Growth Factor, Type 2
  • Receptors, Fibroblast Growth Factor / genetics*
  • Sequence Analysis, DNA
  • Serine / genetics

Substances

  • Receptors, Fibroblast Growth Factor
  • Serine
  • FGFR2 protein, human
  • Receptor Protein-Tyrosine Kinases
  • Receptor, Fibroblast Growth Factor, Type 2
  • Cysteine