Commentary Cellscience Reviews Vol 3 No 3 ISSN 1742-8130

Inactivation of the p53 pathway in retinoblastoma

A recent report from the laboratory of Michael Dyer challenges the long standing belief that retinoblastoma arises from an inherently death resistant cell population and that p53 inactivation is not required for the development of the tumour (Laurie et al 2006). In an elegant study, they show that RB1 deficient retinoblasts undergo p53 mediated apoptosis and exit from the cell cycle. Although mutation of the p53 pathway is not detected in retinoblastomas, they show that subsequent amplification of the MDMX gene and increased expression of the MDMX protein are responsible for suppression of the p53 pathway in the developing RB1 deficient retinoblastoma cells. Our continued understanding the molecular basis for resistance to apoptosis is central for the development of novel approaches for the treatment of metastatic retinoblastoma.

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