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Encyclopedia of Cancer pp 1–5Cite as

Retinoblastoma Protein, Biological and Clinical Functions

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Synonyms

Gene: RB1 – retinoblastoma 1 (including osteosarcoma), RB, OSRC, retinoblastoma tumor suppressor gene, Rb1 (refers to mouse ortholog of human RB1); Protein: pRB – retinoblastoma susceptibility protein, Rb/p105

Definition

pRB is the protein product of the retinoblastoma tumor suppressor gene, RB1. Its biological functions include controlling the cell cycle, preserving genome integrity, mediating differentiation, as well as protecting cells from apoptosis. Mutation of RB1 resulting in functional inactivation of pRB leads to the childhood retinal tumor retinoblastoma and predisposes to osteosarcoma and other cancers. Defective function of pRB or its pathway also contributes to tumor progression in many other cancer types.

Characteristics

The RB1 Gene Encodes the pRB Protein

The RB1 gene contains 27 exons and is located on human chromosome 13q14.2 (NCBI Entrez Gene ID: 5925). The product of RB1is the 110 kDa retinoblastoma susceptibility protein (pRB), composed of 928 amino...

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References

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Author information

Authors and Affiliations

  1. The Hospital for Sick Children, Department of Ophthalmology and Vision Science, The University of Toronto, 555 University Ave, Toronto, ON, M5G 1X8, Canada

    Helen Dimaras & Brenda L. Gallie

Authors
  1. Helen Dimaras
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  2. Brenda L. Gallie
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Corresponding author

Correspondence to Helen Dimaras .

Editor information

Editors and Affiliations

  1. Abt. Tumorgenetik, Deutsches Krebsforschungszentrum, Heidelberg, Germany

    Manfred Schwab

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© 2015 Springer-Verlag Berlin Heidelberg

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Cite this entry

Dimaras, H., Gallie, B.L. (2015). Retinoblastoma Protein, Biological and Clinical Functions. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_5069-9

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_5069-9

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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