Who is the boss in the retinoblastoma family? The point of view of Rb2/p130, the little brother.
This review portrays an updated overview about the possible tumor suppressive properties of the Rb2/p130 gene, the third member of the retinoblastoma (RB) family of genes, including RB itself and p107. After a brief analysis of the established structural and functional similarities among the three genes, the main purpose is to critically analyze present evidence whether Rb2/p130 shares the role of a tumor suppressor. Taking into account the well-proven growth suppressive properties of Rb2/p130 and p107, we discuss the analysis of mutated or deleted forms of Rb2/p130 found in a number of human cancers. Finally, we take into consideration the data provided by the targeted disruption of each RB family gene, alone or in combination, in the mouse model.
Paggi
M G
MG
Laboratory of Cell Metabolism and Pharmacokinetics, Center for Experimental Research, Regina Elena Cancer Institute, 00158 Rome, Italy. paggi@ifo.it
Giordano
A
A
D000818:Animals; D016161:Genes, Retinoblastoma; D006801:Humans; D009369:Neoplasms / Q000235:genetics; D010750:Phosphoproteins / Q000235:genetics*; D011506:Proteins; D016160:Retinoblastoma Protein / Q000235:genetics; D050717:Retinoblastoma-Like Protein p130
D010750:Phosphoproteins; D011506:Proteins; C493837:RBL2 protein, human; D016160:Retinoblastoma Protein; D050717:Retinoblastoma-Like Protein p130