[HTML][HTML] Sickle cell hemoglobin in the ferryl state promotes βCys-93 oxidation and mitochondrial dysfunction in epithelial lung cells (E10)
T Kassa, S Jana, MB Strader, F Meng, Y Jia… - Journal of biological …, 2015 - ASBMB
Polymerization of intraerythrocytic deoxyhemoglobin S (HbS) is the primary molecular event
that leads to hemolytic anemia in sickle cell disease (SCD). We reasoned that HbS may
contribute to the complex pathophysiology of SCD in part due to its pseudoperoxidase
activity. We compared oxidation reactions and the turnover of oxidation intermediates of
purified human HbS and HbA. Hydrogen peroxide (H 2 O 2) drives a catalytic cycle that
includes the following three distinct steps: 1) initial oxidation of ferrous (oxy) to ferryl Hb; 2) …
that leads to hemolytic anemia in sickle cell disease (SCD). We reasoned that HbS may
contribute to the complex pathophysiology of SCD in part due to its pseudoperoxidase
activity. We compared oxidation reactions and the turnover of oxidation intermediates of
purified human HbS and HbA. Hydrogen peroxide (H 2 O 2) drives a catalytic cycle that
includes the following three distinct steps: 1) initial oxidation of ferrous (oxy) to ferryl Hb; 2) …