Molecular characterisation of a mosaicism with a complex chromosome rearrangement: evidence for coincident chromosome healing by telomere capture and neo-telomere formation

J Med Genet. 2007 Apr;44(4):250-6. doi: 10.1136/jmg.2006.045476. Epub 2006 Dec 15.

Abstract

Background: Broken chromosomes must acquire new telomeric "caps" to be structurally stable. Chromosome healing can be mediated either by telomerase through neo-telomere synthesis or by telomere capture.

Aim: To unravel the mechanism(s) generating complex chromosomal mosaicisms and healing broken chromosomes.

Methods: G banding, array comparative genomic hybridization (aCGH), fluorescence in-situ hybridisation (FISH) and short tandem repeat analysis (STR) was performed on a girl presenting with mental retardation, facial dysmorphism, urogenital malformations and limb anomalies carrying a complex chromosomal mosaicism.

Results & discussion: The karyotype showed a de novo chromosome rearrangement with two cell lines: one cell line with a deletion 9pter and one cell line carrying an inverted duplication 9p and a non-reciprocal translocation 5pter fragment. aCGH, FISH and STR analysis enabled the deduction of the most likely sequence of events generating this complex mosaic. During embryogenesis, a double-strand break occurred on the paternal chromosome 9. Following mitotic separation of both broken sister chromatids, one acquired a telomere vianeo-telomere formation, while the other generated a dicentric chromosome which underwent breakage during anaphase, giving rise to the del inv dup(9) that was subsequently healed by chromosome 5 telomere capture.

Conclusion: Broken chromosomes can coincidently be rescued by both telomere capture and neo-telomere synthesis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / embryology
  • Abnormalities, Multiple / genetics*
  • Adolescent
  • Adult
  • Chromatids / genetics
  • Chromatids / ultrastructure
  • Chromosome Banding
  • Chromosome Breakage*
  • Chromosome Deletion*
  • Chromosome Disorders / embryology
  • Chromosome Disorders / genetics*
  • Chromosome Inversion*
  • Chromosomes, Human, Pair 5 / genetics
  • Chromosomes, Human, Pair 5 / ultrastructure*
  • Chromosomes, Human, Pair 9 / genetics
  • Chromosomes, Human, Pair 9 / ultrastructure*
  • Female
  • Gene Duplication
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant, Newborn
  • Intellectual Disability / embryology
  • Intellectual Disability / genetics*
  • Karyotyping
  • Male
  • Microsatellite Repeats
  • Mosaicism*
  • Nucleic Acid Hybridization
  • Telomere / physiology*
  • Translocation, Genetic*