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Retinoblastoma: the paradigm for a genetically inherited cancer syndrome

  • Chapter
Genetic Predisposition to Cancer

Abstract

Genetic analysis of human hereditary cancer has identified the chromosomal location of genes which predispose to tumourigenesis. In the majority of cases it has been shown that loss of function of both alleles of these genes is required for tumour initiation. Because at least one functional copy of the gene product is required to prevent tumour initiation these genes have collectively been called ‘tumour suppressor genes’. Since their normal function is to ensure that differentiation and signal transduction occurs in the appropriate cell type, tumours cannot establish. The first tumour suppressor gene to be isolated was the retinoblastoma gene (RB), predisposing to the children’s eye cancer, retinoblastoma (Rb). The study of this gene has established many of the precedents for the analysis and the cloning of other tumour suppressor genes.

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Authors
  1. John K. Cowell
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Editor information

Editors and Affiliations

  1. Cancer Genetics and Clinical Oncology, Institute of Cancer Research and Royal Marsden Hospital, Surrey, UK

    Rosalind A. Eeles (Senior Lecturer and Honorary Consultant) (Senior Lecturer and Honorary Consultant)

  2. CRC Human Cancer Genetics Research Group, University of Cambridge, UK

    Bruce A. J. Ponder (Director) (Director)

  3. CRC Genetic Epidemiology Unit, University of Cambridge, UK

    Douglas F. Easton (Director) (Director)

  4. Academic Unit of Radiotherapy and Oncology, Institute of Cancer Research and Royal Marsden Hospital, Surrey, UK

    Alan Horwich (Director of Clinical Research) (Director of Clinical Research)

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© 1996 Springer Science+Business Media Dordrecht

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Cowell, J.K. (1996). Retinoblastoma: the paradigm for a genetically inherited cancer syndrome. In: Eeles, R.A., Ponder, B.A.J., Easton, D.F., Horwich, A. (eds) Genetic Predisposition to Cancer. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-4501-3_4

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  • DOI: https://doi.org/10.1007/978-1-4899-4501-3_4

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-412-56580-9

  • Online ISBN: 978-1-4899-4501-3

  • eBook Packages: Springer Book Archive

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