Chromosomal stabilisation by a subtelomeric rearrangement involving two closely related Alu elements

Hum Mol Genet. 1996 Aug;5(8):1163-9. doi: 10.1093/hmg/5.8.1163.

Abstract

We have characterised a subtelomeric rearrangement involving the short arm of chromosome 16 that gives rise to alpha-thalassaemia by deleting the major, remote regulatory element controlling alpha-globin expression. The chromosomal breakpoint lies in an Alu family repeat located only approximately 105 kb from the 16p subtelomeric region. The broken chromosome has been stabilised with a newly positioned telomere acquired by recombination between this 16p Alu element and a closely related subtelomeric Alu element of the Sx subfamily. It seems most likely that this abnormal chromosome has been rescued by the mechanism of telomere capture which may reflect a more general process by which subtelomeric sequences are normally dispersed between chromosomal ends.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Alleles
  • Base Sequence
  • Chromosome Mapping
  • Chromosomes, Human, Pair 16 / genetics*
  • Chromosomes, Human, Pair 16 / ultrastructure
  • DNA / genetics
  • DNA Primers / genetics
  • Female
  • Gene Rearrangement
  • Genotype
  • Globins / genetics
  • Haplotypes
  • Humans
  • In Situ Hybridization, Fluorescence
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Multigene Family
  • Polymerase Chain Reaction
  • Recombination, Genetic
  • Repetitive Sequences, Nucleic Acid*
  • Sequence Homology, Nucleic Acid
  • Telomere / genetics
  • Telomere / ultrastructure
  • alpha-Thalassemia / genetics*

Substances

  • DNA Primers
  • Globins
  • DNA