2024
Hematopoietic Stem Cells Supporting Fetal Erythropoiesis Are Differentially Regulated By Small and Large Ribosomal Subunits
Tang Y, Ling T, Mehmood R, Khan M, Papoin J, Palis J, Steiner L, Durand S, Zon L, Bhoopalan S, Weiss M, Lipton J, Taylor N, Gallagher P, Narla M, Crispino J, Blanc L. Hematopoietic Stem Cells Supporting Fetal Erythropoiesis Are Differentially Regulated By Small and Large Ribosomal Subunits. Blood 2024, 144: 195. DOI: 10.1182/blood-2024-210699.Peer-Reviewed Original ResearchDiamond-Blackfan anemiaHaplo-insufficient miceFetal hematopoiesisHematopoietic stemBFU-EProgenitor cellsCongenital bone marrow failure syndromeMouse modelClinically relevant mouse modelBone marrow failure syndromesProtein haploinsufficiencyBasophilic erythroblastsMarrow failure syndromesBone marrow failureFetal liver cellsRelevant mouse modelConsistent with clinical findingsPost-natal dayConditional mouse modelErythroid lineage cellsRibosomal protein haploinsufficiencyAssociated with mutationsExpression of RUNX1BFU-E stageRUNX1 allele
2023
Rps19 and Rpl5 Play Distinct Roles in hematopoietic Stem Cell maintenance and Erythroid Differentiation
Tang Y, Ling T, Khan M, Rao R, Schulz V, Papoin J, Narla A, Lipton J, Palis J, Steiner L, Gallagher P, Narla M, Crispino J, Blanc L. Rps19 and Rpl5 Play Distinct Roles in hematopoietic Stem Cell maintenance and Erythroid Differentiation. Blood 2023, 142: 144. DOI: 10.1182/blood-2023-189146.Peer-Reviewed Original ResearchFailure of erythropoiesisErythroid differentiationVav-iCreHematopoietic stem cell maintenanceFetal hematopoiesisHematopoietic stemProtein translation ratesStem cell maintenanceRibosomal protein haploinsufficiencyGlobal protein synthesisTerminal erythroid differentiationCell compartmentExpression of RUNX1Stem cell compartmentErythroid fateProgenitor cell compartmentFetal liverRibosome biogenesisPolysome profilingNormal expression levelsRibosomal proteinsProgenitor biologyCell maintenanceScRNAseq studiesTranscription factors
2021
An IDH1-vitamin C crosstalk drives human erythroid development by inhibiting pro-oxidant mitochondrial metabolism
Gonzalez-Menendez P, Romano M, Yan H, Deshmukh R, Papoin J, Oburoglu L, Daumur M, Dumé AS, Phadke I, Mongellaz C, Qu X, Bories PN, Fontenay M, An X, Dardalhon V, Sitbon M, Zimmermann VS, Gallagher PG, Tardito S, Blanc L, Mohandas N, Taylor N, Kinet S. An IDH1-vitamin C crosstalk drives human erythroid development by inhibiting pro-oxidant mitochondrial metabolism. Cell Reports 2021, 34: 108723. PMID: 33535038, PMCID: PMC9169698, DOI: 10.1016/j.celrep.2021.108723.Peer-Reviewed Original ResearchConceptsIsocitrate dehydrogenase 1Oxidative phosphorylationMitochondrial metabolismReactive oxygen speciesHuman erythroid differentiationHuman erythroid developmentMitochondrial oxidative phosphorylationVitamin C homeostasisHSPC developmentIDH1 knockdownErythroid developmentStepwise differentiationErythroid differentiationLate-stage erythropoiesisTerminal stepCritical regulatorHematopoietic stemMitochondrial superoxideMitochondrial oxidationProgenitor cellsDehydrogenase 1Oxygen speciesCongenital dyserythropoietic anemiaCentral roleDyserythropoietic anemia