The Role of Alpha-Hemoglobin Stabilizing Protein in Human Hemoglobin Assembly

摘要: Hemoglobin biosynthesis in erythrocyte precursors involves several steps. The correct ratios and concentrations of normal alpha (α) and beta β) globin proteins must be expressed, apoproteins must be folded correctly, heme must be synthesized and incorporated into these globins, and the resulting α and β subunits must be rapidly and correctly assembled into heterotetramers. These events occur on a large scale in vivo, and dysregulation causes serious clinical disorders such as thalassemia syndromes. Recent work has implicated a conserved erythroid protein known as Alpha-Hemoglobin Stabilizing Protein (AHSP) as a participant in these events. Current evidence suggests that AHSP enhances α subunit stability and diminishes its participation in harmful redox chemistry. There is also evidence that AHSP facilitates one or more early-stage post-translational hemoglobin biosynthetic events. In this work, the rate …