[HTML][HTML] Biophysical characterization of the α-globin binding protein α-hemoglobin stabilizing protein

D Gell, Y Kong, SA Eaton, MJ Weiss… - Journal of Biological …, 2002 - ASBMB
α-Hemoglobin stabilizing protein (AHSP) is a small (12 kDa) and abundant erythroid-specific
protein that binds specifically to free α-(hemo) globin and prevents its precipitation. When
present in excess over β-globin, its normal binding partner, α-globin can have severe
cytotoxic effects that contribute to important human diseases such as β-thalassemia.
Because AHSP might act as a chaperone to prevent the harmful aggregation of α-globin
during normal erythroid cell development and in diseases of globin chain imbalance, it is …