Globin chain synthesis analysis in obligate beta 0-thalassemia heterozygotes with isolated increase of hemoglobin A2 levels

Nouv Rev Fr Hematol (1978). 1981;23(4):193-5.

Abstract

Globin chain synthesis analysis was carried out in 14 obligate beta 0-thalassemia heterozygotes, i.e., parents of children with thalassemia major or intermedia due to homozygous beta 0-thalassemia, with almost normal red blood cell indices and isolated increase of Hb A2 levels. alpha/beta globin chain synthesis ratios were almost balanced or slightly reduced (0.62-1.30). This finding suggests that these heterozygotes carry both alpha- and beta-thalassemia genes. However, alpha-globin structural gene mapping by restriction enzyme analysis must be carried out to confirm this explanation.

Publication types

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

MeSH terms

  • Female
  • Globins / biosynthesis*
  • Hemoglobin A / analysis*
  • Hemoglobin A2 / analysis*
  • Heterozygote
  • Humans
  • Male
  • Thalassemia / blood*
  • Thalassemia / genetics

Substances

  • Globins
  • Hemoglobin A
  • Hemoglobin A2