Impaired binding of AHSP to α chain variants: Hb Groene Hart illustrates a mechanism leading to unstable hemoglobins with α thalassemic like syndrome
C Vasseur-Godbillon, MC Marden, P Giordano… - Blood Cells, Molecules …, 2006 - Elsevier
Alpha hemoglobin stabilizing protein (AHSP) is a small protein of 102 residues induced by
GATA-1, Oct-1-and EKLF. It is synthesized at a high level in the red blood cell precursors
and acts as a chaperone protecting the alpha hemoglobin (α-Hb) chains against
precipitation. AHSP and α-Hb form a heterodimer complex. In the absence of AHSP, α-Hb
oxidizes and precipitates within the erythrocyte precursors of the bone marrow leading to
apoptosis and defective erythropoiesis. In vitro the binding of AHSP to ferrous α-Hb …
GATA-1, Oct-1-and EKLF. It is synthesized at a high level in the red blood cell precursors
and acts as a chaperone protecting the alpha hemoglobin (α-Hb) chains against
precipitation. AHSP and α-Hb form a heterodimer complex. In the absence of AHSP, α-Hb
oxidizes and precipitates within the erythrocyte precursors of the bone marrow leading to
apoptosis and defective erythropoiesis. In vitro the binding of AHSP to ferrous α-Hb …