title: Tissue-specific tumor suppressor activity of retinoblastoma gene homologs p107 and p130.
abstract: The retinoblastoma gene family consists of three genes: RB, p107, and p130. While loss of pRB causes retinoblastoma in humans and pituitary gland tumors in mice, tumorigenesis in other tissues may be suppressed by p107 and p130. To test this hypothesis, we have generated chimeric mice from embryonic stem cells carrying compound loss-of-function mutations in the Rb gene family. We found that Rb/p107- and Rb/p130-deficient mice were highly cancer prone. We conclude that in a variety of tissues tumor development by loss of pRB is suppressed by its homologs p107 and p130. The redundancy of the retinoblastoma proteins in vivo is reflected by the behavior of Rb-family-defective mouse embryonic fibroblasts in vitro.
authors.affiliation: Division of Molecular Biology, The Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands.
authors.forename: Jan-Hermen
authors.initials: JH
authors.lastname: Dannenberg
authors.forename: Leontine
authors.initials: L
authors.lastname: Schuijff
authors.forename: Marleen
authors.initials: M
authors.lastname: Dekker
authors.forename: Martin
authors.initials: M
authors.lastname: van der Valk
authors.forename: Hein
authors.initials: H
authors.lastname: te Riele
mesh_terms: D000818:Animals; D015139:Blotting, Southern; D002678:Chimera; D051379:Mice; D009687:Nuclear Proteins / Q000235:genetics / Q000502:physiology*; D016133:Polymerase Chain Reaction; D011506:Proteins / Q000235:genetics / Q000502:physiology*; D050720:Retinoblastoma-Like Protein p107; D050717:Retinoblastoma-Like Protein p130
chemical_list: D009687:Nuclear Proteins; D011506:Proteins; C493838:Rbl2 protein, mouse; D050720:Retinoblastoma-Like Protein p107; D050717:Retinoblastoma-Like Protein p130