Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Retinoblastoma Protein, Cellular Biochemistry

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_5070-2

Definition

Retinoblastoma protein is a 110 kDa nuclear phosphoprotein (tumor suppression). The gene locus maps to 13q14.1–q14.2. Retinoblastoma is an embryonic neoplasm of retinal origin. Young children with a germline mutation in one Rb1 allele have a 95 % chance of developing a retinoblastoma tumor in their eyes. Mutation in the Rb1 allele also predisposes patients to develop other tumors, such as osteosarcomas and fibrosarcomas. Almost two-thirds of the secondary tumors arising in patients with retinoblastoma are mesenchymal in origin. Most mutations in Rb1 lead to premature termination of translation.

Characteristics

pRb and Its Family Members

The pRb, p107, and p130 proteins form the “pocket protein” family that is crucial in cell-cycleregulation. pRb and p107/p130 have five domains of high conservation. pRb shares between 30 % and 35 % sequence identity with p107/p130. Most of the conserved sequences lie within the so-called pocket region (amino acid 379–792), which is composed...

Keywords

Pocket Protein Transcription Activation Domain Pocket Region Growth Suppression Function Tumor Suppression Function 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Brehm A, Kouzarides T (1999) Retinoblastoma protein meets chromatin. Trends Biochem Sci 24:142–145CrossRefPubMedGoogle Scholar
  2. Dyson N (1998) The regulation of E2F by pRB-family proteins. Genes Dev 12:2245–2262CrossRefPubMedGoogle Scholar
  3. Helin K (1998) Regulation of cell proliferation by the E2F transcription factors. Curr Opin Genet Dev 8:28–35CrossRefPubMedGoogle Scholar
  4. La Thangue NB (1994) DRTF1/E2F: an expanding family of heterodimeric transcription factors implicated in cell-cycle control. Trends Biochem Sci 19:108–114CrossRefPubMedGoogle Scholar
  5. Mulligan G, Jacks T (1998) The retinoblastoma gene family: cousins with overlapping interests. Trends Genet 14:223–229CrossRefPubMedGoogle Scholar
  6. Weinberg RA (1995) The retinoblastoma protein and cell-cycle control. Cell 81:323–330CrossRefPubMedGoogle Scholar
  7. White R (1997) Regulation of RNA polymerase I and III by retinoblastoma protein: a mechanism for growth control? Trends Biochem Sci 22:77–80CrossRefPubMedGoogle Scholar
  8. Whyte P (1995) The retinoblastoma protein and its relatives. Semin Cancer Biol 6:83–90CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Division of Biochemistry and Molecular Biology, Davidson BuildingUniversity of GlasgowGlasgowUK
  2. 2.Department of OncologyUniversity of OxfordOxfordUK