Retinoblastoma protein family in cell cycle and cancer: A review

Marco G. Paggi, Alfonso Baldi, Francesco Bonetto, Antonio Giordano

Research output: Contribution to journalArticlepeer-review

Abstract

Two genes, p107 and Rb2/p130, are strictly related to RB, the most investigated tumor suppressor gene, responsible for susceptibility to retinoblastoma. The products of these three genes, namely pRb, p107, and pRb2/p130 are characterized by a peculiar steric conformation, called 'pocket,' responsible for most of the functional interactions characterizing the activity of these proteins in the homeostasis of the cell cycle. The interest in these genes and proteins springs from their ability to regulate cell cycle processes negatively, being able, for example, to dramatically slow down neoplastic growth. So far, among these genes, only RB is firmly established to act as a tumor suppressor, because its lack-of-function is clearly involved in tumor onset and progression. It has been found deleted or mutated in most retinoblastomas and sarcomas, but its inactivation is likely to play a crucial role in other types of human cancers. The two other members of the family have been discovered more recently and are currently under extensive investigation. We review analogies and differences among the pocket protein family members, in an attempt to understand their functions in normal and cancer cells.

Original languageEnglish
Pages (from-to)418-430
Number of pages13
JournalJournal of Cellular Biochemistry
Volume62
Issue number3
DOIs
Publication statusPublished - Sep 1 1996

Keywords

  • cell cycle
  • p107
  • pocket protein
  • Rb2/p130
  • retinoblastoma gene
  • tumor suppressor genes

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology

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