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Human Plasma and Recombinant Hemopexins: Heme Binding Revisited
Review

Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease

1
Department of Anesthesiology, Center for Translational Research in Neurodegenerative Disease, University of Florida College of Medicine, Gainesville, FL 32610, USA
2
Department of Biobehavioral Nursing Science, University of Florida College of Nursing, Gainesville, FL 32610, USA
3
Departments of Neurology, Psychiatry, Pharmaceutics, and Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL 32610, USA
*
Author to whom correspondence should be addressed.
Academic Editor: William T. Morgan
Int. J. Mol. Sci. 2021, 22(12), 6408; https://doi.org/10.3390/ijms22126408
Received: 21 May 2021 / Revised: 9 June 2021 / Accepted: 13 June 2021 / Published: 15 June 2021
(This article belongs to the Special Issue Heme and Hemopexin: Multitasking Molecules)
Circulating hemopexin is the primary protein responsible for the clearance of heme; therefore, it is a systemic combatant against deleterious inflammation and oxidative stress induced by the presence of free heme. This role of hemopexin is critical in hemolytic pathophysiology. In this review, we outline the current research regarding how the dynamic activity of hemopexin is implicated in sickle cell disease, which is characterized by a pathological aggregation of red blood cells and excessive hemolysis. This pathophysiology leads to symptoms such as acute kidney injury, vaso-occlusion, ischemic stroke, pain crises, and pulmonary hypertension exacerbated by the presence of free heme and hemoglobin. This review includes in vivo studies in mouse, rat, and guinea pig models of sickle cell disease, as well as studies in human samples. In summary, the current research indicates that hemopexin is likely protective against these symptoms and that rectifying depleted hemopexin in patients with sickle cell disease could improve or prevent the symptoms. The data compiled in this review suggest that further preclinical and clinical research should be conducted to uncover pathways of hemopexin in pathological states to evaluate its potential clinical function as both a biomarker and therapy for sickle cell disease and related hemoglobinopathies. View Full-Text
Keywords: heme; heme binding protein; hemoglobinopathy; hemolysis; inflammation; immune modulation; oxidative stress; sickle cell anemia; vaso-occlusion; therapy heme; heme binding protein; hemoglobinopathy; hemolysis; inflammation; immune modulation; oxidative stress; sickle cell anemia; vaso-occlusion; therapy
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MDPI and ACS Style

Ashouri, R.; Fangman, M.; Burris, A.; Ezenwa, M.O.; Wilkie, D.J.; Doré, S. Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease. Int. J. Mol. Sci. 2021, 22, 6408. https://doi.org/10.3390/ijms22126408

AMA Style

Ashouri R, Fangman M, Burris A, Ezenwa MO, Wilkie DJ, Doré S. Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease. International Journal of Molecular Sciences. 2021; 22(12):6408. https://doi.org/10.3390/ijms22126408

Chicago/Turabian Style

Ashouri, Rani, Madison Fangman, Alicia Burris, Miriam O. Ezenwa, Diana J. Wilkie, and Sylvain Doré. 2021. "Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease" International Journal of Molecular Sciences 22, no. 12: 6408. https://doi.org/10.3390/ijms22126408

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