The p53 gene plays a crucial role in the control of cell growth and is frequently inactivated by mutations in cancer cells. Differently from most tumor types, p53 mutations in neuroblastoma (NB) are rare suggesting that p53 status is irrelevant in the pathogenesis of this tumor. The biological functions of p53 in NB, however, are inhibited by the accumulation of the protein in the cytoplasm. Moreover, after DNA damage, p53 translocates to the nucleus but its capacity to induce G1 cell-cycle arrest is attenuated.1,2,3

A considerable interest in NB research was recently raised by the discovery of p73, a p53 homologue located at 1p36.3, which transactivates several p53 target genes, induces apoptosis and inhibits cell proliferation. The contribution of p73 to tumor suppression however, is still unclear.4 p73 is a direct downstream effector of E2F-1 and is an essential component of the p53-independent apoptotic pathway. In developing sympathetic neurons of mice, p73 is predominantly expressed as a truncated anti-apoptotic isoform (ΔNp73), lacking the transactivation domain of the full length protein (TAp73). ΔNp73 antagonizes p53 and suppresses the transactivating activity of TAp73 by oligomerization and competition for DNA binding.5,6

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