The retinoblastoma tumour suppressor in development and cancer

  title={The retinoblastoma tumour suppressor in development and cancer},
  author={Marie Classon and Ed Harlow},
  journal={Nature Reviews Cancer},
Since its discovery, the retinoblastoma (RB) tumour-suppressor protein has been a focal point of cancer research. Accumulating evidence indicates a complex role for RB in cell proliferation, differentiation and survival. To further complicate matters, proteins that are related to RB have redundant as well as antagonistic functions. Recent studies of knockout mice and cells that lack one or more of these proteins have begun to clarify their various context-specific functions and the unique… 

Cellular mechanisms of tumour suppression by the retinoblastoma gene

The retinoblastoma (RB) tumour suppressor gene is functionally inactivated in a broad range of paediatric and adult cancers, and a plethora of cellular functions and partners have been identified for

Tailoring to RB: tumour suppressor status and therapeutic response

The retinoblastoma tumour suppressor is a crucial regulator of cell-cycle progression that is invoked in response to a myriad of anti-mitogenic signals and its status may be particularly informative in directing treatment regimens.

The retinoblastoma protein--from bench to bedside.

The search for the retinoblastoma cell of origin

Retinoblastoma, a rare childhood cancer of the retina that is caused by RB inactivation, is a good model in which to search for a tumour cell of origin, because retinal development is well understood and the initiating genetic lesion is well characterized.

The Retinoblastoma Tumour Suppressor

Which functions of pRB contribute most to its tumour-suppressor activity are determined, why certain tumours select against RB but others do not, and what kind of additional mutations are likely to cooperate with RB loss in cancer as a function of tissue type are discussed.

Dysfunction of the RB Retinoblastoma Gene in Cancer

Emerging evidence shows that RB status can influence the response to different anti-cancer therapeutics according to the context, suggesting that a thorough understanding of all RB functions in cancer is more crucial than ever.

Retinoblastoma and tumor-suppressor gene therapy.

  • Hong‐Ji Xu
  • Medicine, Biology
    Ophthalmology clinics of North America
  • 2003

Role of the retinoblastoma tumor suppressor protein in cellular differentiation

The increasing evidence for a role of pRb105 in cellular differentiation and the fact that various cancers, which contain mutant pR b105, or mutations in proteins in the pRB105 pathway, are perhaps a result of deregulation of differentiation are discussed.

Retinoblastoma family proteins: insights gained through genetic manipulation of mice

The phenotypic consequences of R b family ablation in mice are summarized, and how these findings contribute to the increasingly complex picture of Rb family function in development and tumor suppression are discussed.



The retinoblastoma gene family in differentiation and development

The retinoblastoma (Rb) tumor suppressor gene and its close relatives p107 and p130 are best known for their function in the control of cell cycle progression. In recent years, however, a new role

Effects of an Rb mutation in the mouse

A mouse strain has been constructed in which one allele of Rb is disrupted, and heterozygous animals are not predisposed to retinoblastoma, but some display pituitary tumours arising from cells in which the wild-type Rb allele is absent.

The retinoblastoma gene family: cousins with overlapping interests.

Suppression of the neoplastic phenotype by replacement of the RB gene in human cancer cells.

This demonstration of suppression of the neoplastic phenotype by a single gene provides direct evidence for an essential role of the RB gene in tumorigenesis.

Retinoblastoma protein partners.

The role of p53 and pRB in apoptosis and cancer.

Cooperative tumorigenic effects of germline mutations in Rb and p53

The phenotypic effects of combined germline mutations in these two tumour suppressor genes Rb and p53 are described to indicate that mutations in Rband p53 can cooperate in the transformation of certain cell types in the mouse.

Paradoxical increase in retinoblastoma protein in colorectal carcinomas may protect cells from apoptosis.

  • H. YamamotoJ. Soh I. Weinstein
  • Biology, Medicine
    Clinical cancer research : an official journal of the American Association for Cancer Research
  • 1999
The findings suggest that the increased expression of pRb in colorectal carcinoma cells may provide a homeostatic mechanism that protects them from growth inhibition and apoptosis, perhaps by counterbalancing potentially toxic effects of excessive E2F activity.

Retinoblastoma protein meets chromatin.

Retinoblastoma in transgenic mice

It is reported that expression of a viral oncogene, the simian virus 40 T antigen, in the retina of transgenic mice produces heritable ocular tumours with histological, ultrastructural and immunohis-tochemical features identical to those of human retinoblastoma.