Tissue-specific tumor suppressor activity of retinoblastoma gene homologs p107 and p130
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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.
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Targeted changes in upstream RB pathway components can inactivate all Rb family genes.
“Targeted alterations in upstream components of the RB pathway can result in the inactivation of all of the Rb family genes, but because the RB pathway is not strictly linear, these experiments might not directly inform us about the mechanisms of RB tumour suppressive action4,7,150–153.”
From Cellular mechanisms of tumour suppression by the retinoblastoma gene (2008) · cites this paper as [150]
The RB pathway is not strictly linear.
“Targeted alterations in upstream components of the RB pathway can result in the inactivation of all of the Rb family genes, but because the RB pathway is not strictly linear, these experiments might not directly inform us about the mechanisms of RB tumour suppressive action4,7,150–153.”
From Cellular mechanisms of tumour suppression by the retinoblastoma gene (2008) · cites this paper as [150]
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References 71 · Cited by 137
- Authors
- Dannenberg, J.-H., Schuijff, L., Dekker, M., van der Valk, M., & Riele, H. T.
- Affiliations
- The Netherlands Cancer Institute (NL)
- Venue
- Genes & Development
- Publisher
- Cold Spring Harbor Laboratory
- Published
- 2004-12-01
- Volume
- 18
- Issue
- 23
- Pages
- 2952-2962
- ISSN
- 0890-9369 (ISSN-L)
- 1549-5477
- PMC
- PMC534655
- OpenAlex
- W2131488073
- Cited by
- 137
- References
- 71
- Citations by year
- 2026: 1 · 2025: 5 · 2024: 3 · 2023: 5 · 2022: 2 · 2021: 3 · 2020: 4 · 2019: 2 · 2018: 5 · 2017: 3 · 2016: 5 · 2015: 7 · 2014: 3 · 2013: 7 · 2012: 18
- Type
- posted content
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Genes & Development (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Oncology › Cancer-related Molecular Pathways
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Chromatin Remodeling and Cancer
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinal Development and Disorders
- Keywords
- p107, p130, retinoblastoma protein, tumor suppressor activity, chimeric mice, cancer susceptibility, protein redundancy, mouse embryonic fibroblasts
- MeSH terms
- Animals, Chimera, Nuclear Proteins, Proteins, Blotting, Southern, Polymerase Chain Reaction, Retinoblastoma-Like Protein p130, Retinoblastoma-Like Protein p107, Mice
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