Oncogenic Intra-p53 Family Member Interactions in Human Cancers - PubMed
- ️Fri Jan 01 2016
Review
Oncogenic Intra-p53 Family Member Interactions in Human Cancers
Maria Ferraiuolo et al. Front Oncol. 2016.
Abstract
The p53 gene family members p53, p73, and p63 display several isoforms derived from the presence of internal promoters and alternative splicing events. They are structural homologs but hold peculiar functional properties. p53, p73, and p63 are tumor suppressor genes that promote differentiation, senescence, and apoptosis. p53, unlike p73 and p63, is frequently mutated in cancer often displaying oncogenic "gain of function" activities correlated with the induction of proliferation, invasion, chemoresistance, and genomic instability in cancer cells. These oncogenic functions are promoted either by the aberrant transcriptional cooperation of mutant p53 (mutp53) with transcription cofactors (e.g., NF-Y, E2F1, Vitamin D Receptor, Ets-1, NF-kB and YAP) or by the interaction with the p53 family members, p73 and p63, determining their functional inactivation. The instauration of these aberrant transcriptional networks leads to increased cell growth, low activation of DNA damage response pathways (DNA damage response and DNA double-strand breaks response), enhanced invasion, and high chemoresistance to different conventional chemotherapeutic treatments. Several studies have clearly shown that different cancers harboring mutant p53 proteins exhibit a poor prognosis when compared to those carrying wild-type p53 (wt-p53) protein. The interference of mutantp53/p73 and/or mutantp53/p63 interactions, thereby restoring p53, p73, and p63 tumor suppression functions, could be among the potential therapeutic strategies for the treatment of mutant p53 human cancers.
Keywords: apoptosis; differentiation; gain of function; homology; isoforms; p53 gene family members; protein–protein interaction; target genes.
Figures

p53, p73, and p63 isoforms obtained by the presence of internal promoters in the sequence (ΔN isoforms) and by alternative splicing. The N-terminal transcription-activation domain (TAD) contains two subdomains (AD1 and AD2) and is the binding-site for regulators of gene transcription; in p63 and p73 proteins, the C-terminal transactivation inhibitory domain (TID) binds to the TAD preventing constitutive transcriptional activity. The Proline-rich sequence recognition domains (PRD) can recognize proline-rich motifs of interacting proteins and has been reported to be essential for the induction of apoptosis driven by p53, p73 and p63. The DNA-binding domain (DBD) is responsible for the binding to DNA consensus of target genes and the oligomerization domain (OD) enables monomers assembly in active oligomers. The sterile alpha motif domain (SAMD) of p63 and p73 is arranged in a small five-helix bundle and is involved in protein–protein interactions. The basic region (BR) in the C-terminal of p53 is involved in the control of the DNA binding affinity.

The presence of mutant p53 moves the tumor suppressor functions of p73 and p63 to an oncogenic outcome through binding and inactivating p73 and p63 transcriptional activity. The development of new anticancer strategies, such as increasing p73/p63 activities and employing molecules that interfere with mutantp53/p73 and/or mutantp53/p63 interactions [e.g., the molecule p53 reactivation and induction of massive apoptosis (PRIMA-1Met), short interfering mutp53 peptides (SIMPs), NSC59984 and Aptamers], could restore the p53 family’s tumor suppressor functions.
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