[HTML][HTML] α-Hemoglobin stabilizing protein (AHSP), a kinetic scheme of the action of a human mutant, AHSPV56G
T Brillet, V Baudin-Creuza, C Vasseur… - Journal of biological …, 2010 - ASBMB
A kinetic analysis has been made of the interaction of α-Hb chains with a mutant α-
hemoglobin stabilizing protein, AHSP V56G, which is the first case of an AHSP mutation
associated with clinical symptoms of mild thalassemia syndrome. The chaperone AHSP is
thought to protect nascent α chains until final binding to the partner β-Hb. Rather than
protecting α chains, the mutant chaperone is partially unfolded but recovers its secondary
structure via interaction with α-Hb. For both AHSP WT and AHSP V56G, the binding to α-Hb …
hemoglobin stabilizing protein, AHSP V56G, which is the first case of an AHSP mutation
associated with clinical symptoms of mild thalassemia syndrome. The chaperone AHSP is
thought to protect nascent α chains until final binding to the partner β-Hb. Rather than
protecting α chains, the mutant chaperone is partially unfolded but recovers its secondary
structure via interaction with α-Hb. For both AHSP WT and AHSP V56G, the binding to α-Hb …
