The genetics of thalassemia

Schweiz Med Wochenschr. 1983 Oct 8;113(40):1415-9.

Abstract

Two newly developed techniques have greatly enlarged our knowledge of the basic genetic defects of the beta-thalassemias: 1) Restriction enzyme digestion of cellular DNA followed by analysis of the cleaved fragments. 2) Cloning of beta-globin genes from patients with beta-thalassemia and subsequent determination of the nucleotide sequence of these genes. Some of the results are presented. Only rarely a deletion of the beta-globin gene was found. In most cases, a mutation within the beta-globin gene was discovered as basis for the beta-thalassemia syndrome. These and other findings are a starting point for planning a therapy of beta-thalassemia by means of gene technology: correction of the defect within the abnormal beta-globin gene, replacement of the beta-globin gene, stimulation of gamma-globin synthesis.

Publication types

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

MeSH terms

  • DNA Restriction Enzymes
  • DNA, Recombinant
  • Genetic Carrier Screening
  • Globins / biosynthesis
  • Globins / genetics
  • Humans
  • Mutation
  • Protein Biosynthesis
  • Thalassemia / classification
  • Thalassemia / genetics*
  • Thalassemia / therapy

Substances

  • DNA, Recombinant
  • Globins
  • DNA Restriction Enzymes