Article
Alpha-hemoglobin stabilizing protein (AHSP), a kinetic scheme of the action of a human mutant, AHSPV56G.
The Journal of biological chemistry - 4 Jun 2010
Brillet Thomas, Baudin-Creuza Véronique, Vasseur Corinne, Domingues-Hamdi Elisa, Kiger Laurent, Wajcman Henri, Pissard Serge, Marden Michael C
Abstract excerpt
A kinetic analysis has been made of the interaction of alpha-Hb chains with a mutant alpha-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 alpha chains until final binding to the partner beta-Hb. Rather than protecting alpha chains, the mutant chaperone is...
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