2024
Dexamethasone Targets E2F4 to Induce Erythroid Progenitor Renewal
Papoin J, Schulz V, Khan F, Barnes B, Lipton J, Steiner L, Narla M, Gallagher P, Blanc L. Dexamethasone Targets E2F4 to Induce Erythroid Progenitor Renewal. Blood 2024, 144: 1076. DOI: 10.1182/blood-2024-211687.Peer-Reviewed Original ResearchDiamond-Blackfan anemiaCD34+ cellsErythroid progenitorsSteroid responsivenessExpression of E2F4Peripheral bloodBone marrowE2F4 expressionDEX treatmentCultured CD34+ cellsInherited bone marrow failure syndromeMechanisms of steroid responseSide effectsBone marrow failure syndromesDiamond-Blackfan anemia patientsBone marrow erythroid progenitorsIncreased expressionMechanisms of corticosteroid actionAssociated with side effectsIncreased risk of infectionMarrow failure syndromesResponse to steroidsGene promoterSerum-free culture systemMarrow erythroid progenitorsEndometriosis in a 22-Year-Old with Premature Ovarian Insufficiency Secondary to Pre-Pubertal Bone Marrow Transplant: a Case Report
Liao C, Ramani S, Parkash V, Kodaman P. Endometriosis in a 22-Year-Old with Premature Ovarian Insufficiency Secondary to Pre-Pubertal Bone Marrow Transplant: a Case Report. Reproductive Sciences 2024, 31: 2700-2705. PMID: 38806998, DOI: 10.1007/s43032-024-01601-z.Peer-Reviewed Original ResearchPremature ovarian insufficiencyBone marrow transplantationProgressive dysmenorrheaPelvic painMarrow transplantationContraceptive pillsInherited bone marrow failure syndromeBone marrow failure syndromesSuperficial endometriotic lesionsChronic pelvic painMarrow failure syndromesOral contraceptive pillsCases of endometriosisDiamond-Blackfan anemiaReproductive age womenSpontaneous ovarian activityCyclic progestinNulligravid femaleWorsening arthralgiasTransdermal estrogenDiagnostic laparoscopyGonadotoxic chemotherapyOral estradiolHormone therapyOvarian insufficiencyNormal Erythroid Precursors in Diamond-Blackfan Anemia: A Rare Case Highlighting Challenges That Remain
Prior D, Sowa A, Pashankar F. Normal Erythroid Precursors in Diamond-Blackfan Anemia: A Rare Case Highlighting Challenges That Remain. Journal Of Pediatric Hematology/Oncology 2024, 46: e195-e198. PMID: 38277626, DOI: 10.1097/mph.0000000000002820.Peer-Reviewed Original ResearchConceptsDiamond-Blackfan anemiaDiamond-BlackfanDiagnosis of Diamond-Blackfan anemiaInherited bone marrow failure syndromeBone marrow failure syndromesMarrow failure syndromesDiagnostically challenging casesFailure syndromeAtypical manifestationsSpontaneous remissionRare caseErythroid precursorsChallenging casesGenetic etiologyPhenotypic manifestationsAnemiaPatientsRemissionRelapseHypoplasiaSyndromeEtiologyDiagnosis
2023
SATB1 Chromatin Loops Regulate Megakaryocyte/Erythroid Progenitor Expansion by Facilitating HSP70 and GATA1 Induction
Wilkes M, Chae H, Scanlon V, Cepika A, Wentworth E, Saxena M, Eskin A, Chen Z, Glader B, Roncarolo M, Nelson S, Sakamoto K. SATB1 Chromatin Loops Regulate Megakaryocyte/Erythroid Progenitor Expansion by Facilitating HSP70 and GATA1 Induction. Stem Cells 2023, 41: 560-569. PMID: 36987811, PMCID: PMC10267687, DOI: 10.1093/stmcls/sxad025.Peer-Reviewed Original ResearchConceptsMegakaryocyte/erythroid progenitorsSpecial AT-rich sequence-binding protein 1Diamond-Blackfan anemiaInherited bone marrow failure syndromeChromatin loopsInduction of HSP70Hsp70 geneShock protein 70Progenitor expansionBiological functionsEarly myeloid progenitorsBone marrow failure syndromesErythroid factorsErythroid progenitorsMyeloid progenitorsMarrow failure syndromesProtein 1Protein 70Blackfan anemiaHSP70Specific sitesProgenitorsCell modelSATB1 expressionInduction3189 – REVISITING THE HEMATOPOIETIC AND ERYTHROPOIETIC DEFECTS IN RPS19 AND RPL5 HAPLOINSUFFICIENCY AT THE DEVELOPMENTAL LEVEL
Tang Y, Ling T, Durand S, Palis J, Steiner L, Mohandas N, Gallagher P, Lipton J, Crispino J, Blanc L. 3189 – REVISITING THE HEMATOPOIETIC AND ERYTHROPOIETIC DEFECTS IN RPS19 AND RPL5 HAPLOINSUFFICIENCY AT THE DEVELOPMENTAL LEVEL. Experimental Hematology 2023, 124: s144. DOI: 10.1016/j.exphem.2023.06.296.Peer-Reviewed Original ResearchRibosomal proteinsDiamond-Blackfan anemiaGlobal protein synthesisTerminal erythroid differentiationStem cell exhaustionHematopoietic stem cell exhaustionKey transcription factorInherited bone marrow failure syndromeFailure of erythropoiesisCell cycle arrestHematopoietic developmentMutant cellsTranscription factorsProgenitor stageCRISPR/Erythroid differentiationVav-iCreMendelian ratioDefective erythropoiesisRPS19Bone marrow failure syndromesLoxP sitesProtein synthesisBone marrow failureHematopoietic progenitors
2022
Downregulation of SATB1 by miRNAs reduces megakaryocyte/erythroid progenitor expansion in preclinical models of Diamond–Blackfan anemia
Wilkes MC, Scanlon V, Shibuya A, Cepika AM, Eskin A, Chen Z, Narla A, Glader B, Roncarolo MG, Nelson SF, Sakamoto KM. Downregulation of SATB1 by miRNAs reduces megakaryocyte/erythroid progenitor expansion in preclinical models of Diamond–Blackfan anemia. Experimental Hematology 2022, 111: 66-78. PMID: 35460833, PMCID: PMC9255422, DOI: 10.1016/j.exphem.2022.04.005.Peer-Reviewed Original ResearchConceptsMegakaryocyte/erythroid progenitorsDiamond-Blackfan anemiaRibosomal proteinsPathogenesis of DBAErythroid progenitorsHuman hematopoietic stemInherited bone marrow failure syndromeChromatin regulatorsRibosomal insufficiencyErythroid differentiationProgenitor expansionBone marrow failure syndromesCancer predispositionHematopoietic stemMiR-30MiR-34Molecular pathwaysMegakaryocyte expansionMarrow failure syndromesNormal erythropoiesisProgenitor cellsCoordinated actionSATB1ProgenitorsDifferentiation
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