RB1: a prototype tumor suppressor and an enigma
- Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, Massachusetts 02129, USA
- Corresponding author:dyson{at}helix.mgh.harvard.edu
Abstract
The retinoblastoma susceptibility gene (RB1) was the first tumor suppressor gene to be molecularly defined.RB1 mutations occur in almost all familial and sporadic forms of retinoblastoma, and this gene is mutated at variable frequencies in a variety of other human cancers. Because of its early discovery, the recessive nature ofRB1 mutations, and its frequency of inactivation,RB1 is often described as a prototype for the class of tumor suppressor genes. Its gene product (pRB) regulates transcription and is a negative regulator of cell proliferation. Although these general features are well established, a precise description of pRB's mechanism of action has remained elusive. Indeed, in many regards, pRB remains an enigma. This review summarizes some recent developments in pRB research and focuses on progress toward answers for the three fundamental questions that sit at the heart of the pRB literature: What does pRB do? How does the inactivation of RB change the cell? How can our knowledge of RB function be exploited to provide better treatment for cancer patients?
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Article is online athttp://www.genesdev.org/cgi/doi/10.1101/gad.282145.116.
Freely available online through theGenes & Development Open Access option.
This article, published inGenes & Development, is available under a Creative Commons License (Attribution 4.0 International), as described athttp://creativecommons.org/licenses/by/4.0/.
