Inactivation of the p53 pathway in retinoblastoma

  title={Inactivation of the p53 pathway in retinoblastoma},
  author={Nikia A. Laurie and Stacy L. Donovan and Chie-Schin Shih and Jiakun Zhang and Nicholas L Mills and Christine Fuller and Amina F. A. S. Teunisse and Suzanne Lam and Yolande F M Ramos and Adithi Mohan and Dianna Johnson and Matthew K. Wilson and Carlos Rodriguez-Galindo and Micaela Quarto and Sarah Francoz and Susan M. Mendrysa and R. Kiplin Guy and J. -C. Marine and Aart G. Jochemsen and Michael A. Dyer},
Most human tumours have genetic mutations in their Rb and p53 pathways, but retinoblastoma is thought to be an exception. Studies suggest that retinoblastomas, which initiate with mutations in the gene retinoblastoma 1 (RB1), bypass the p53 pathway because they arise from intrinsically death-resistant cells during retinal development. In contrast to this prevailing theory, here we show that the tumour surveillance pathway mediated by Arf, MDM2, MDMX and p53 is activated after loss of RB1 during… CONTINUE READING
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