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Publication : Li-Fraumeni syndrome: a p53 family affair.

First Author  Iwakuma T Year  2005
Journal  Cell Cycle Volume  4
Issue  7 Pages  865-7
PubMed ID  15917654 Mgi Jnum  J:101109
Mgi Id  MGI:3590607 Doi  10.4161/cc.4.7.1800
Citation  Iwakuma T, et al. (2005) Li-Fraumeni syndrome: a p53 family affair. Cell Cycle 4(7):865-7
abstractText  The p53 alterations frequently found in human tumors are missense mutations in the DNA binding domain. These p53 mutations have been shown to have gain-of-function or dominant-negative properties in multiple experiments. The consequences of these p53 mutations at physiological levels on the development of a tumor were unclear. Using mouse models, three recent papers have shed light on the mechanisms of mutant p53 and its family members, p63 and p73, in tumorigenesis. Interestingly, the p53 point mutant mice had a similar phenotype to p53 family compound mutant mice suggesting that there is an interplay between the p53 family members in tumorigenesis and Li-Fraumeni syndrome.
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