Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease
ContextSickle cell disease is characterized by a state of nitric oxide resistance and limited
bioavailability of L-arginine, the substrate for nitric oxide synthesis. We hypothesized that
increased arginase activity and dysregulated arginine metabolism contribute to endothelial
dysfunction, pulmonary hypertension, and patient outcomes. ObjectiveTo explore the role of
arginase in sickle cell disease pathogenesis, pulmonary hypertension, and mortality.
DesignPlasma amino acid levels, plasma and erythrocyte arginase activities, and pulmonary …
bioavailability of L-arginine, the substrate for nitric oxide synthesis. We hypothesized that
increased arginase activity and dysregulated arginine metabolism contribute to endothelial
dysfunction, pulmonary hypertension, and patient outcomes. ObjectiveTo explore the role of
arginase in sickle cell disease pathogenesis, pulmonary hypertension, and mortality.
DesignPlasma amino acid levels, plasma and erythrocyte arginase activities, and pulmonary …