Sudanese (δβ)0-Thalassemia: Identification and Characterization of a Novel 9.6 kb Deletion

Hemoglobin. 2015;39(5):368-70. doi: 10.3109/03630269.2015.1057736. Epub 2015 Jul 8.

Abstract

We report a case of δβ-thalassemia (δβ-thal) trait in an adult male originally from Sudan. Multiplex ligation-dependent probe amplification (MLPA) was used to localize the approximate boundaries of the deletion, followed by polymerase chain reaction (PCR) amplification and sequence analysis of the junction fragment to determine the precise deletion endpoints. The deletion spans 9594 bp, with the 5' deletion endpoint located 1560 bp upstream of the δ-globin gene and the 3' endpoint within the second intervening sequence (IVS-II) of the β-globin gene.

Keywords: (δβ)0-thalassemia [(δβ)0-thal]; Hb F silencer region; Sudanese; deletion.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Base Sequence
  • DNA Mutational Analysis
  • Genotype
  • Humans
  • Introns
  • Male
  • Mutation*
  • Phenotype
  • Sequence Deletion
  • Sudan
  • beta-Globins / chemistry
  • beta-Globins / genetics*
  • beta-Thalassemia / diagnosis*
  • beta-Thalassemia / genetics*
  • delta-Globins / chemistry
  • delta-Globins / genetics*
  • delta-Thalassemia / diagnosis*
  • delta-Thalassemia / genetics*

Substances

  • beta-Globins
  • delta-Globins

Supplementary concepts

  • Delta-Beta Thalassemia