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Association between the oligomeric status of p53 and clinical outcomes in Li-Fraumeni syndrome

Journal of the National Cancer Institute Jan 17, 2019

Fischer NW, et al. - Investigators assessed data to analyze the association between the oligomeric status of p53 and clinical consequences in Li-Fraumeni syndrome (LFS), a rare hereditary cancer disorder. They reported the quaternary structure of p53, an important determinant influencing the cellular functions and clinical consequences of LFS candidates. They noted a loss of p53 oligomerization associated with clinical results. They observed carriers of monomeric p53 mutants showed complete penetrance, with a 2.11-fold greater risk of cancer-related death and a statistically significantly lower median survival age in comparison to carriers of multimeric p53 mutants. The association between p53 oligomeric state and clinical phenotype implied that TP53 mutations were not all equivalent and recommended the implementation of new genotype-adapted guidelines for the management of LFS cases with TP53 mutations in the oligomerization domain.
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