Familial Mediterranean fever gene mutations in north-eastern part of Anatolia with special respect to rare mutations

Gene. 2015 Sep 1;568(2):170-5. doi: 10.1016/j.gene.2015.05.045. Epub 2015 May 21.

Abstract

We aimed to determine the frequency of mutations, carrier rates and the association of rare mutations with Familial Mediterranean Fever (FMF) symptoms. There is a need to evaluate as many different populations as possible in order to determine either specific rare mutations or a range of disease-associated mutations. The demographic data and FMF symptoms related to MEFV gene mutations were collected from 731 participants. Exon 2 and exon 10 of the MEFV gene were tested by DNA sequencing. The rare mutations were identified as: M694I (1.1%, n=12), E148V (0.6%, n=6), T267I (0.5%, n=5), L110P (0.2%, n=2), E167D (0.2%, n=2), K695R (0.1%, n=1) and an insertion G (Guanine) mutation (0.4%, n=4) at the 777th codon of exon 10. We used routine comprehensive detection systems such as Sanger sequence that can catch rare mutations, for definite diagnosis and treatment of FMF disease.

Keywords: DNA sequencing; FMF; MEFV; Rare mutation.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Cytoskeletal Proteins / genetics*
  • DNA Mutational Analysis
  • Familial Mediterranean Fever / genetics*
  • Female
  • Gene Frequency
  • Genetic Association Studies
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Mutation, Missense
  • Pyrin
  • Turkey
  • Young Adult

Substances

  • Cytoskeletal Proteins
  • MEFV protein, human
  • Pyrin