Haploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells.

@article{Dutt2011HaploinsufficiencyFR,
  title={Haploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells.},
  author={Shilpee Dutt and Anupama Narla and Katherine Lin and Ann Mullally and Nirmalee Abayasekara and Christine Megerdichian and Frederick H. Wilson and Treeve Currie and Arati Khanna-Gupta and Nancy Berliner and Jeffery L. Kutok and Benjamin L. Ebert},
  journal={Blood},
  year={2011},
  volume={117 9},
  pages={2567-76}
}
Haploinsufficiency for ribosomal protein genes has been implicated in the pathophysiology of Diamond-Blackfan anemia (DBA) and the 5q-syndrome, a subtype of myelodysplastic syndrome. The p53 pathway is activated by ribosome dysfunction, but the molecular basis for selective impairment of the erythroid lineage in disorders of ribosome function has not been determined. We found that p53 accumulates selectively in the erythroid lineage in primary human hematopoietic progenitor cells after… CONTINUE READING