SNH-119014, a novel pyruvate kinase activator, enhances ATP production and reduces oxidative stress in erythroid cells from patients with β-thalassemia major

BackgroundThalassemia is a common disease worldwide. Oxidative stress contributes to ineffective erythropoiesis and hemolysis in β-thalassemia major (β-TM). Increasing cellular adenosine triphosphate (ATP) production by activating the activity of the pyruvate kinase (PK) may counteract oxidative stress. SNH-119014 is a novel allosteric PK activator targeting the same protein as mitapivat (AG-348), a first-in-class agent known to ameliorate anemia in thalassemia. This study aim to evluate the eff