Alpha Hemoglobin Stabilizing Protein (AHSP) Protein

Background

Alpha Hemoglobin Stabilizing Protein (AHSP) is a molecular chaperone that specifically binds to free alpha-globin subunits during erythropoiesis, stabilizing them and preventing their aggregation or precipitation. This function is critical for maintaining the proper stoichiometry of alpha- and beta-globin chains required for the assembly of functional hemoglobin tetramers. AHSP also protects alpha-globin from oxidative damage by maintaining it in a reduced state. Dysregulation or deficiency of AHSP is associated with hematological disorders such as beta-thalassemia, where excess alpha-globin chains lead to oxidative stress and ineffective erythropoiesis. AHSP is highly expressed in erythroid precursor cells, and its expression is tightly regulated during red blood cell development. Therapeutic approaches that modulate AHSP levels or function are being explored as potential treatments for thalassemias and related hemoglobinopathies.