Conditional haploinsufficiency of the retinoblastoma tumor suppressor gene.
Recent work demonstrates that retention of a single functional retinoblastoma susceptibility (RB1) allele is insufficient to maintain genome stability. Haploinsufficiency of RB1 accelerates cancer pathogenesis in concert with inactivation of tumor protein p53. Collectively, multiple lines of evidence suggest revision of the '2-hit' model to include conditional haploinsufficiency of RB1.
London Regional Cancer Program Western University; London, Ontario, Canada; Department of Biochemistry; Western University; London, Ontario, Canada.
Charles A
CA
Ishak
London Regional Cancer Program Western University; London, Ontario, Canada; Children's Health Research Institute Western University; London, Ontario, Canada; Department of Biochemistry; Western University; London, Ontario, Canada.
Frederick A
FA
Dick
chromosome instability; loss of heterozygosity; p53; replication stress; tumor suppressor gene