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The mRNA-Binding Protein IGF2BP1 Restores Fetal Hemoglobin in Cultured Erythroid Cells from Patients with β-Hemoglobin Disorders

Christopher B. Chambers, Jeffrey Gross, Katherine L. Pratt, Xiang Guo, Colleen Byrnes, Y. Terry Lee, Donald Lavelle, Ann Dean, Jeffery L. Miller, Andrew Wilber

2020Molecular Therapy — Methods & Clinical Development25 citationsDOIOpen Access PDF

Abstract

cells revealed that IGF2BP1-dependent HbF induction could ameliorate the chain imbalance in β-thalassemia or potently suppress expression of sickle β-globin in SCD. In all cases, fetal γ-globin mRNA increased and adult β-globin decreased due, in part, to formation of contacts between the locus control region (LCR) and γ-globin genes. We conclude that expression of IGF2BP1 in adult erythroid cells has the potential to maximize HbF expression in patients with severe β-hemoglobin disorders by reversing the developmental γ- to β-globin switch.

Topics & Concepts

HemoglobinFetal hemoglobinMessenger RNAMolecular biologyBiologyFetusChemistryCell biologyBiochemistryGeneticsGenePregnancyHemoglobinopathies and Related DisordersRNA modifications and cancerBlood groups and transfusion
The mRNA-Binding Protein IGF2BP1 Restores Fetal Hemoglobin in Cultured Erythroid Cells from Patients with β-Hemoglobin Disorders | Litcius