Skin tumors Rb(eing) uncovered

The Rb1 gene was the first bona fide tumor suppressor identified and cloned more than 25 years ago. Since then, a plethora of studies have revealed the functions of pRb and the existence of a sophisticated and strictly regulated pathway that modulates such functional roles. An emerging paradox affec...

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Main Authors: CLOTILDE eCOSTA, Jesus M Paramio, MIRENTXU eSANTOS
Format: Article
Language:English
Published: Frontiers Media S.A. 2013-12-01
Series:Frontiers in Oncology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00307/full
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author CLOTILDE eCOSTA
Jesus M Paramio
MIRENTXU eSANTOS
author_facet CLOTILDE eCOSTA
Jesus M Paramio
MIRENTXU eSANTOS
author_sort CLOTILDE eCOSTA
collection DOAJ
description The Rb1 gene was the first bona fide tumor suppressor identified and cloned more than 25 years ago. Since then, a plethora of studies have revealed the functions of pRb and the existence of a sophisticated and strictly regulated pathway that modulates such functional roles. An emerging paradox affecting Rb1 in cancer connects the relatively low number of mutations affecting Rb1 gene in specific human tumors, compared with the widely functional inactivation of pRb in most, if not in all, human cancers. The existence of a retinoblastoma family of proteins pRb, p107 and p130 and their potential unique and overlapping functions as master regulators of cell cycle progression and transcriptional modulation by similar processes, may provide potential clues to explain such conundrum. Here, we will review the development of different genetically engineered mouse models, in particular those affecting stratified epithelia, and how they have offered new avenues to understand the roles of the Rb family members and their targets in the context of tumor development and progression.
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spelling doaj.art-389af188b77341d2a1404e70332f39ca2022-12-21T18:37:53ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2013-12-01310.3389/fonc.2013.0030767007Skin tumors Rb(eing) uncoveredCLOTILDE eCOSTA0Jesus M Paramio1MIRENTXU eSANTOS2Centro de Investigaciones Energéticas Medioambientales y teconológicas (CIEMAT)Centro de Investigaciones Energéticas Medioambientales y teconológicas (CIEMAT)Centro de Investigaciones Energéticas Medioambientales y teconológicas (CIEMAT)The Rb1 gene was the first bona fide tumor suppressor identified and cloned more than 25 years ago. Since then, a plethora of studies have revealed the functions of pRb and the existence of a sophisticated and strictly regulated pathway that modulates such functional roles. An emerging paradox affecting Rb1 in cancer connects the relatively low number of mutations affecting Rb1 gene in specific human tumors, compared with the widely functional inactivation of pRb in most, if not in all, human cancers. The existence of a retinoblastoma family of proteins pRb, p107 and p130 and their potential unique and overlapping functions as master regulators of cell cycle progression and transcriptional modulation by similar processes, may provide potential clues to explain such conundrum. Here, we will review the development of different genetically engineered mouse models, in particular those affecting stratified epithelia, and how they have offered new avenues to understand the roles of the Rb family members and their targets in the context of tumor development and progression.http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00307/fullE2F Transcription FactorsEpidermismouse modelsTransgenic micep53 pathwaypRb
spellingShingle CLOTILDE eCOSTA
Jesus M Paramio
MIRENTXU eSANTOS
Skin tumors Rb(eing) uncovered
Frontiers in Oncology
E2F Transcription Factors
Epidermis
mouse models
Transgenic mice
p53 pathway
pRb
title Skin tumors Rb(eing) uncovered
title_full Skin tumors Rb(eing) uncovered
title_fullStr Skin tumors Rb(eing) uncovered
title_full_unstemmed Skin tumors Rb(eing) uncovered
title_short Skin tumors Rb(eing) uncovered
title_sort skin tumors rb eing uncovered
topic E2F Transcription Factors
Epidermis
mouse models
Transgenic mice
p53 pathway
pRb
url http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00307/full
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