Linked Life Data

8344484

Source:http://linkedlifedata.com/resource/pubmed/id/8344484

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
1993-9-9
pubmed:abstractText
Studies of retinoblastoma clearly identify mutation of the RB1 gene on chromosome 13 as the primary cause of this cancer. However, all retinoblastoma tumors have an abnormal karyotype (1, 2) indicating the presence of additional mutations and suggesting that mutation of both RB1 alleles is insufficient for development of retinoblastoma. In addition, analysis of RB1 expression and of RB1 mutations in different tumors leads to the following dilemma: while the RB1 gene product, p110RB1, is expressed in most dividing cells, germline mutations inactivating the function of p110RB1 predispose primarily to retinoblastoma and to a lesser extent to osteosarcoma, but do not predispose to cancer in general. However, many tumors contain somatic mutations that disrupt RB1 function. Thus, we are faced with the unusual situation in which germline mutations in the RB1 gene predispose to a very limited set of cancers, but somatic mutations in RB1 appear to contribute to malignancy in many tissues. We propose that the role of the RB1 gene is to maintain the cells in a stable, quiescent state required for terminal differentiation and that the effect of RB1 mutations in different tissues depends on the pattern of differentiation in that tissue. In tissues where differentiation follows a linear process from undifferentiated precursors to fully differentiated cells, loss of RB1 function during early stages of differentiation may lead to uncontrolled growth and the development of cancer. On the other hand, in cell renewal systems where cell number is usually maintained by a process of programmed cell death (PCD) or apoptosis, loss of RB1 function may lead to cell death.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0892-6638
pubmed:author
pubmed:issnType
Print
pubmed:volume
7
pubmed:geneSymbol
RB1
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
846-54
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1993
pubmed:articleTitle
Speculations on the roles of RB1 in tissue-specific differentiation, tumor initiation, and tumor progression.
pubmed:affiliation
Division of Immunology and Cancer Research, Hospital for Sick Children, Toronto, Ontario, Canada.
pubmed:publicationType
Journal Article, Review, Research Support, Non-U.S. Gov't