Article
Association Between the Oligomeric Status of p53 and Clinical Outcomes in Li-Fraumeni Syndrome.
Journal of the National Cancer Institute - 1 Dec 2018
Fischer Nicholas W, Prodeus Aaron, Tran James, Malkin David, Gariépy Jean
Abstract excerpt
Li-Fraumeni syndrome (LFS) is a rare hereditary cancer disorder with highly variable clinical outcomes that results from germline mutations in the TP53 gene. Here we report that the quaternary structure of p53 is an important factor affecting cellular functions and the clinical outcomes of LFS patients (n = 87). Specifically, carriers of monomeric p53 mutants (n = 56) exhibited complete penetrance, with a...
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