The role of the retinoblastoma gene in tumour pathogenesis

The retinoblastoma gene (RB-1) was originally identified as the gene involved in hereditary retinoblastoma. However, RB-1 mutations are found in a number of common mesenchymal and epithelial malignancies. The retinoblastoma protein (pRB) acts as a transcriptional regulator of genes involved in DNA synthesis and cell-cycle control. In this regard, the functional interaction between pRB and the E2F transcription factor family appears to be critical. The pRB-E2F interaction is, in turn, regulated by a pathway that includes cyclin D1, cdk4, and p16. Mutations that affect this pathway have been documented in nearly every type of adult cancer. Thus, perturbation of pRB function may be required for the development of cancer. Insights into the biochemical functions of pRB, and its upstream regulators, may form the basis for the development of novel antineoplastic agents.

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Experimental Model to Study the Role of Retinoblastoma Gene Product (pRb) for Determination of Adipocyte Differentiation

Bulletin of Experimental Biology and Medicine ◽

10.1007/s10517-015-2944-3 ◽

2015 ◽

Vol 159 (2) ◽

pp. 289-292 ◽

Cited By ~ 2

Author(s):

B. V. Popov ◽

P. S. Shilo ◽

O. V. Zhidkova ◽

A. M. Zaichik ◽

N. S. Petrov

Keyword(s):

Experimental Model ◽

Gene Product ◽

Adipocyte Differentiation ◽

Retinoblastoma Gene ◽

Retinoblastoma Gene Product

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An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma

Sarcoma ◽

10.1080/13577140500229596 ◽

2005 ◽

Vol 9 (3-4) ◽

pp. 151-156 ◽

Cited By ~ 5

Author(s):

J. K. O'Neill ◽

C. A. Stone ◽

P. Sarsfield ◽

M. Smith ◽

S. F. Smithson ◽

...

Keyword(s):

Surgical Management ◽

Metastatic Potential ◽

Upper Extremities ◽

Low Grade ◽

Retinoblastoma Gene ◽

Marginal Excision ◽

Lipomatous Tumour ◽

Well Differentiated ◽

Grade Tumour

Well differentiated liposarcoma (atypical lipomatous tumour) is a low grade tumour, with no metastatic potential unless dedifferentiation supervenes. When superficial, it recurs locally only occasionally after marginal excision. We present a patient in whom bilateral childhood retinoblastoma was followed by later development of massive confluent areas of low grade liposarcoma and lipomatous tissue affecting the upper extremities and trunk. We discuss the role of mutations in the retinoblastoma gene (RB1) in linking these conditions and demonstrate the surgical management of an extremely unusual and challenging case.

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