Is FGF13 a major contributor to genetic epilepsy with febrile seizures plus?

Epilepsy Res. 2016 Dec:128:48-51. doi: 10.1016/j.eplepsyres.2016.10.008. Epub 2016 Oct 25.

Abstract

Mutation of fibroblast growth factor 13 (FGF13) has recently been implicated in genetic epilepsy with febrile seizures plus (GEFS+) in a single family segregating a balanced translocation with a breakpoint in this X chromosome gene, predicting a partial knockout involving 3 of 5 known FGF13 isoforms. Investigation of a mouse model of complete Fgf13 knock-out revealed increased susceptibility to hyperthermia-induced seizures and epilepsy. Here we investigated whether mutation of FGF13 would explain other cases of GEFS+ compatible with X-linked inheritance. We screened the coding and splice site regions of the FGF13 gene in a sample of 45 unrelated probands where GEFS+ segregated in an X-linked pattern. We subsequently identified a de novo FGF13 missense variant in an additional patient with febrile seizures and facial edema. Our data suggests FGF13 is not a common cause of GEFS+.

Keywords: FGF13; GEFS+; Sequencing.

MeSH terms

  • Child, Preschool
  • Cohort Studies
  • Edema / genetics
  • Epilepsy, Generalized / genetics*
  • Face
  • Female
  • Fibroblast Growth Factors / genetics*
  • Genes, X-Linked
  • Genetic Testing
  • Humans
  • Male
  • Mutation, Missense
  • Phenotype
  • SOXF Transcription Factors / genetics
  • Seizures, Febrile / genetics*

Substances

  • SOX18 protein, human
  • SOXF Transcription Factors
  • fibroblast growth factor 13
  • Fibroblast Growth Factors