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Hematol Oncol Clin North Am. 2014 Apr;28(2):301-21. doi: 10.1016/j.hoc.2013.11.008.

Alterations of the arginine metabolome in sickle cell disease: a growing rationale for arginine therapy.

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  • 1Division of Emergency Medicine, Department of Pediatrics, Emory-Children's Center for Cystic Fibrosis and Airways Disease Research, Emory University School of Medicine, 1645 Tullie Circle Northeast, Atlanta, GA 30329, USA. Electronic address:


Low global arginine bioavailability (GAB) is associated with numerous complications of SCD including early mortality. Mechanisms of arginine dysregulation involve a complex paradigm of excess activity of the arginine-consuming enzyme arginase, elevated levels of asymmetric dimethylarginine, altered intracellular arginine transport, and nitric oxide synthase dysfunction. Restoration of GAB through exogenous supplementation is therefore, a promising therapeutic target. Studies of arginine therapy demonstrate efficacy in treating patients with leg ulcers, pulmonary hypertension risk, and pain. Co-administration with hydroxyurea increases levels of nitrite and fetal hemoglobin. Addressing the alterations in the arginine metabolome may result in new strategies for treatment of SCD.

Copyright © 2014 Elsevier Inc. All rights reserved.


Arginase; Arginine; Hemolysis; Nitric oxide; Sickle cell disease; Vasoocclusive pain episodes

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