Role of Alpha Hemoglobin Stabilizing Protein Expression in Beta Thalassemia Patients

Abstract

Background: Beta (β )-Thalassemia is an autosomal recessive inherited disease causing variable degrees of anemia. The molecular defects are owing to point mutations or small deletions leading to diminution or absence of β -globin chain synthesis. The unmatched α -Hb is destructive to itself and other cellular components, resulting in ineffective erythropoiesis and hemolysis. Alpha-Hemoglobin Stabilizing Protein (AHSP) is a scavenger protein of erythroid that fastly and reversibly attach the α subunit of hemoglobin molecule preventing its precipitation and subsequent hemolysis.