Mitapivat (AG-348) hydrochloride is an orally active and selective allosteric pyruvate kinase R ( PK-R ) activator. Mitapivat hydrochloride accelerates the reaction of PK-R catalyzing the conversion of phosphoenolpyruvate to pyruvate, thereby promoting the glycolytic pathway, increasing the production of adenosine triphosphate (ATP) in red blood cells, and reducing the level of 2,3-diphosphoglycerate (2,3-DPG). Mitapivat hydrochloride is promising for research of pyruvate kinase deficiency and other anemia-related diseases.
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