New euchromatic variant dup(11)(p15.3p15.1) transmitted through two generations defined by low coverage whole genome sequencing

Am J Med Genet A. 2021 Oct;185(10):3053-3056. doi: 10.1002/ajmg.a.62357. Epub 2021 May 27.

Abstract

We report on a 14-year old boy, his father, and his paternal uncle, all three carriers of a duplication of chromosomal region 11p15.3-p15.1. The aberration was transmitted by the grandmother, who is carrier of a balanced insertion 46,XX,ins(14;11)(q32.1;p15.3p15.1). In order to determine the precise molecular basis of this structural variant, we performed low-coverage whole genome sequencing on the boy's father. This approach allowed precise determination of the genomic breakpoints and revealed a duplication of 6.9 Mb, centromeric to the Beckwith-Wiedemann/Silver-Russell syndrome critical region in 11p15.5, that inserted in inverse orientation into 14q32.12 (according to HGVS nomenclature: NC_000014.8:g.92871000_92871001ins[NC_000011.9:g.12250642_19165928inv;T]). To our knowledge, this is the first report of a duplication of 11p15.3-p15.1 involving more than 40 genes and transmitted through two generations without apparent clinical effects.

Keywords: chromosomal insertion; directly transmitted unbalanced chromosome abnormalities; low-coverage whole genome sequencing.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Beckwith-Wiedemann Syndrome / genetics*
  • Beckwith-Wiedemann Syndrome / pathology
  • Chromosomes, Human, Pair 11 / genetics
  • Chromosomes, Human, Pair 14 / genetics
  • Genetic Predisposition to Disease*
  • Genome, Human / genetics
  • Humans
  • Male
  • Pedigree
  • Silver-Russell Syndrome / genetics*
  • Silver-Russell Syndrome / pathology
  • Translocation, Genetic / genetics*
  • Whole Genome Sequencing