A constitutional mutation within the retinoblastoma gene detected by PFGE

Clin Genet. 1994 Jan;45(1):5-10. doi: 10.1111/j.1399-0004.1994.tb03981.x.

Abstract

Retinoblastoma may be caused by constitutional mutations in the retinoblastoma gene which segregates as an autosomal dominant inherited predisposition for developing retinoblastoma tumours. Since 75% of these cases are new mutations, there is a need for methods to identify carriers of such germ-line mutations, so that informed genetic counselling is available to patients and close relatives. We have used pulsed-field gel electrophoresis in screening 20 unrelated cases with bilateral retinoblastoma. One constitutional mutation could be detected, and was found to be caused by a balanced chromosome (4;13) translocation with the breakpoint within intron 17 of the retinoblastoma gene.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chromosomes, Human, Pair 13*
  • Chromosomes, Human, Pair 4*
  • DNA Restriction Enzymes
  • Electrophoresis, Gel, Pulsed-Field
  • Genes, Retinoblastoma / genetics*
  • Genetic Markers
  • Humans
  • Mutation*
  • Translocation, Genetic*
  • Tumor Cells, Cultured

Substances

  • Genetic Markers
  • DNA Restriction Enzymes