Molecular characterization of G6PD deficiency in Cyprus

Blood Cells Mol Dis. 2004 Jul-Aug;33(1):25-30. doi: 10.1016/j.bcmd.2004.03.004.

Abstract

In the present study, we determined the frequency of glucose-6-phosphate dehydrogenase (G6PD) deficiency in Cyprus using two different procedures in two separate adult population groups: a semiquantitative fluorescence test on blood spotted on filter paper and a quantitative spectrophotometric test on liquid blood. The frequency of G6PD deficiency among healthy adult males was found to be 5.1% using the semiquantitative procedure and 6.4% using the quantitative procedure. Neither method was able to detect all the expected female heterozygotes (5.3% and 47.1% of the expected number, respectively). A total of 21 male hemizygotes, 1 female homozygote and 9 female heterozygotes that tested positive for G6PD deficiency were studied at the molecular level. All 32 chromosomes were genotyped and five different mutations were identified. The Mediterranean mutation in exon 6 (563C-->T) (Ser188Phe) was found to be the most common variant in the Cypriot population, accounting for 52.6% of the deficient alleles. In the remaining chromosomes, four different mutations were identified: three known mutations, Kaiping 1388G-->A (Arg463His), Chatham 1003G-->A (Ala335Thr) and Acrokorinthos 463C-->G (His155Asp), and one previously undescribed mutation in exon 3, 148C-->T (Pro50Ser), which we called G6PD Kambos. We conclude that the frequency of G6PD deficiency in Cypriot males is 6.4%, and that this deficiency is the result of several different mutations. Although all the individuals carrying the Mediterranean variant can be detected using a semiquantitative screening method, a quantitative enzyme measurement is required to detect the G6PD variants with less severe enzyme deficiencies, while the most appropriate method for heterozygote detection is DNA analysis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Clinical Laboratory Techniques / standards
  • Cyprus / epidemiology
  • DNA Mutational Analysis
  • Female
  • Genotype
  • Glucosephosphate Dehydrogenase Deficiency / diagnosis*
  • Glucosephosphate Dehydrogenase Deficiency / epidemiology*
  • Glucosephosphate Dehydrogenase Deficiency / genetics
  • Humans
  • Male
  • Mass Screening / methods
  • Molecular Epidemiology
  • Mutation, Missense
  • Prevalence
  • Spectrum Analysis