Spontaneous Abnormalities in Normal Fibroblasts from Patients with Li-Fraumeni Cancer Syndrome: Aneuploidy and Immortalization

摘要: Abstract Families of patients with the Li-Fraumeni cancer syndrome have an inherited pattern sarcomas and various other types cancers that follow a dominant mode transmission, early age onset, exhibit multiple primary tumors. As soft tissue (including fibrosarcomas) are frequently observed this syndrome, in vitro growth characteristics fibroblasts derived from skin biopsies were studied. Control maintained normal morphology eventually senesced culture. Fibroblasts seven eight affected individuals developed changes morphology, anchor-age-independent growth, chromosomal abnormalities. In fashion similar to donors they underwent crisis during which their was slow, but continued grow past point at control samples had stopped dividing (35 population doublings). escape senescence, growing well beyond 35 doublings rates early-passage cells. Patient maintain transformed cell remain nontumorigenic nude mice. These observations behavior may predictive value for determination gene carriers within these families who high risk cancer.