Beta-thalassemia intermedia due to two novel mutations in the promoter region of the beta-globin gene

Eur J Haematol. 2008 Apr;80(4):346-50. doi: 10.1111/j.1600-0609.2007.01017.x. Epub 2007 Dec 10.

Abstract

Two novel beta-thalassemia mutations affecting the promoter region of the beta-globin gene are described. The first mutation, found in a Moroccan family, is a G-->A substitution at position -190 relative to the beta-globin gene Cap site. The second, found in an Algerian patient, is a G-->C substitution at position -56 relative to the beta-globin Cap site. These two mutations occur in a region (-50 to -300) where promoter elements important for differential control of gene expression have been described and lead to beta-thalassemia intermedia in association with a beta(0)-thalassemia mutations.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Female
  • Gene Expression Regulation
  • Globins / genetics*
  • Humans
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Mutation / genetics*
  • Promoter Regions, Genetic / genetics*
  • beta-Thalassemia / genetics*

Substances

  • Globins