Haploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells
Dutt S, Narla A, Lin K, Mullally A, Abayasekara N, Megerdichian C, Wilson FH, Currie T, Khanna-Gupta A, Berliner N, Kutok JL, Ebert BL. Haploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells. Blood 2010, 117: 2567-2576. PMID: 21068437, PMCID: PMC3062351, DOI: 10.1182/blood-2010-07-295238.Peer-Reviewed Original ResearchMeSH KeywordsAnemia, Diamond-BlackfanAnemia, MacrocyticAnimalsBenzothiazolesCell CycleCell LineageCell NucleolusChromosome DeletionChromosomes, Human, Pair 5Cyclin-Dependent Kinase Inhibitor p21Erythroid Precursor CellsHaploinsufficiencyHematopoiesisHumansImidazolesMiceMice, Inbred BALB CMyelodysplastic SyndromesPiperazinesProtein BindingProto-Oncogene Proteins c-mdm2Ribosomal ProteinsRNA, Small InterferingTolueneTumor Suppressor Protein p53ConceptsErythroid progenitor cellsDiamond-Blackfan anemiaMyelodysplastic syndromeProgenitor cellsInduction of p53Bone marrow biopsyErythroid lineageNuclear p53 stainingFailure of erythropoiesisBone marrow failureHuman erythroid progenitor cellsMarrow biopsyHematopoietic progenitor cellsP53 stainingHuman hematopoietic progenitor cellsCell cycle arrestPharmacologic inhibitionMarrow failureNutlin-3Consequent cell cycle arrestP53 pathwayCycle arrestSelective impairmentExpression of shRNAsP53