Cathepsin B, a Negative Regulator of Autophagy, Identified As a Novel Therapeutic Drug Target in Sickle Cell Disease (original) (raw)

Blood, 2020

Abstract

Sickle cell disease (SCD) is an inherited blood disorder that affects millions of people worldwide. The disease is caused by a mutation of the beta-globin gene that results in polymerization of the sickle hemoglobin (HbS) when deoxygenated. Reactive oxygen species (ROS) induced hemolysis is a major critical event in SCD. We have previously shown that abnormal retention of mitochondria in the erythrocytes of both SCD patients and SCD mice is associated with elevated ROS levels and hemolysis (Exp. Hem.2017;50:46-52). The mechanism responsible for mitochondrial retention in SCD is unknown. Autophagy is one of the processes responsible for the elimination of mitochondria during erythroid differentiation. Autophagy is a conserved physiological process that promotes cellular homeostasis through the recycling of proteins, protein aggregates, and removal of damaged organelles. Hypothesis: In this study, we have investigated the hypothesis that autophagy pathway dysregulation is responsible ...

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