Increased oxidative metabolism is associated with erythroid precursor expansion in β0-thalassaemia/Hb E disease

α-Hemoglobin-stabilizing Protein (AHSP) Perturbs the Proximal Heme Pocket of Oxy-α-hemoglobin and Weakens the Iron-Oxygen Bond

“…Oxidative damage occurs in erythroid cells of ␤-thalassaemic mice that contain elevated levels of unpaired ␣Hb (21)(22)(23)(24). Kong et al (6) showed similar increases in reactive oxygen species in AHSP Ϫ/Ϫ mice, suggesting that AHSP has a role in limiting reactive oxygen species production during normal erythropoiesis.…”

miR-214 protects erythroid cells against oxidative stress by targeting ATF4 and EZH2

“…Therefore, erythroid cells are highly sensitive to oxidative stress, and aberrant ROS accumulation incurs impaired erythropoiesis, shortened lifespan of RBCs and even hemolysis [66,69,70]. A comprehensive protective machinery is employed in erythroid cells during differentiation.…”

Review: Beta-thalassemia and molecular chaperones

“…Some of the mechanisms reported to date include a reduction of cell size, condensation of chromatin material and nuclear degradation in the erythroid cell, which resembles the apoptosis [7]. Previous studies have shown that beta-thal patients have a higher number of erythroid progenitors, basophilic and polychromatophilic erythroblasts, but the lower number of orthochromatic erythroblasts compared to healthy individuals [23][24][25][26][27][28].…”

“…The ineffective erythropoiesis in beta-thal is reported to be characterized by increased differentiation of the erythroid precursors [13], maturation arrest at the polychromatophilic stage and also increased levels of apoptosis in the erythroid progenitors [23][24][25][26][27][28]. Increased apoptosis is an essential feature of ineffective erythropoiesis.…”

“…Increased apoptosis is an essential feature of ineffective erythropoiesis. The exact mechanism(s) associated with deposition of the free alpha-globin chains or synthesis of reactive oxygen species (ROS) leading to increased levels of apoptosis in beta-thal patients remains to be identified [27].…”