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Article

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

10 Nov 2010

Abstract excerpt

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...

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PMID: 21068437
DOI: 10.1182/blood-2010-07-295238

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