Multiplex ligation-dependent probe amplification identification of 17 different beta-globin gene deletions (including four novel mutations) in the UK population

Hemoglobin. 2009;33(6):406-16. doi: 10.3109/03630260903344564.

Abstract

Large deletions of the beta-globin gene cluster are problematic to diagnose, and consequently the frequency and range of these mutations in the UK is unknown. Here we present a study evaluating the efficacy of the recently available technique of multiplex ligation-dependent prob amplification (MLPA) to determine the range and frequency of these deletions in the UK population. The results revealed a large deletion mutation in 75 of 316 patient samples collected over a 3-year period. Of these, 52 had a common (deltabeta)(0)-thalassemia [(deltabeta)(0)-thal] or hereditary persistence of fetal hemoglobin (HPFH) allele and 23 had rare or novel deletions resulting in (epsilon(G)gamma(A)gammadeltabeta)(0)-thal, (G)gamma(A)gamma(deltabeta)(0)-thal and beta(0)-thal. A total of 17 different deletions were found, 10 of which were rare and four were most likely novel [Asian Indian (epsilon(G)gamma(A)gammadeltabeta)(0)-thal, African (deltabeta)(0)-thal, African beta(0)-thal and Afghanistani beta(0)-thal]. The MLPA technique detected examples from all four categories of beta-globin gene deletions and demonstrated the wide molecular basis of deletional beta-thal/HPFH in UK patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Fetal Hemoglobin / genetics
  • Gene Deletion*
  • Gene Frequency
  • Hemoglobinopathies / epidemiology
  • Hemoglobinopathies / ethnology
  • Hemoglobinopathies / genetics
  • Hemoglobins, Abnormal / genetics*
  • Humans
  • Mutation*
  • Nucleic Acid Amplification Techniques / methods*
  • Thalassemia / genetics
  • United Kingdom / epidemiology
  • United Kingdom / ethnology
  • beta-Globins / genetics*

Substances

  • Hemoglobins, Abnormal
  • beta-Globins
  • Fetal Hemoglobin