[New beta0-thalassaemic insertion mutation (CD 7/8, +G) in a Slovak family, associated with the Mediterranean haplotype IX]

Vnitr Lek. 1999 Mar;45(3):151-4.
[Article in Czech]

Abstract

The authors describe a newly identified beta0-thalassaemic mutation found in two subjects from two generations of a Slovak family. The beta0-thalassaemic allele developed by insertion of one nucleotide (+G, CD 7/8) into the first exon of the beta-globin gene. The mutation causes a shift of the open globin reading frame which leads to the development of a terminal codon in codon 22. The thalassaemic allele is associated with the mediterranean haplotype IX. The mutation has in both heterozygotes the phenotype of beta0-thalassaemia minor with a slightly elevated level of HbF.

Publication types

  • Case Reports
  • English Abstract
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Codon, Terminator / genetics
  • Exons / genetics
  • Female
  • Frameshift Mutation*
  • Globins / genetics*
  • Haplotypes
  • Heterozygote
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Phenotype
  • Sequence Analysis, DNA
  • beta-Thalassemia / genetics*

Substances

  • Codon, Terminator
  • Globins