AHSP Background Information å-hemoglobin stabilizing protein (AHSP), also designated erythroid associated factor (ERAF), is an erythroid-specific protein that acts as a chaperone to prevent the aggregation of å-hemoglobin during normal erythroid cell development. It specifically protects free å-hemoglobin from precipitation in live cells and in solution. It forms a heterodimer with free å-hemoglobin, but not with ∫-hemoglobin or hemoglobin A (å2-∫-2). AHSP localizes to the cytoplasm and is expressed in the blood and bone marrow. The AHSP protein is downregulated in transmissible spongiform encephalopathies (TSEs). AHSP may regulate pathological states of å-hemoglobin excess such as ∫-thalassemia, a group of hereditary disorders involving the decreased production of normal adult hemoglobin (HbA) that are characterized by a deficiency in the synthesis of ∫-globin chains.
