TY - JOUR
T1 - Retinoblastoma protein family in cell cycle and cancer
T2 - A review
AU - Paggi, Marco G.
AU - Baldi, Alfonso
AU - Bonetto, Francesco
AU - Giordano, Antonio
PY - 1996/9/1
Y1 - 1996/9/1
N2 - 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.
AB - 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.
KW - cell cycle
KW - p107
KW - pocket protein
KW - Rb2/p130
KW - retinoblastoma gene
KW - tumor suppressor genes
UR - http://www.scopus.com/inward/record.url?scp=0029739541&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029739541&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1097-4644(199609)62:3<418::AID-JCB12>3.0.CO;2-E
DO - 10.1002/(SICI)1097-4644(199609)62:3<418::AID-JCB12>3.0.CO;2-E
M3 - Article
C2 - 8872613
AN - SCOPUS:0029739541
VL - 62
SP - 418
EP - 430
JO - Journal of Cellular Biochemistry
JF - Journal of Cellular Biochemistry
SN - 0730-2312
IS - 3
ER -