Sickle cell disease: role of oxidative stress and antioxidant therapy

R Vona, NM Sposi, L Mattia, L Gambardella, E Straface… - Antioxidants, 2021 - mdpi.com
Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb),
which affects approximately a million people worldwide. It is characterized by a single …

The role of RBC oxidative stress in sickle cell disease: from the molecular basis to pathologic implications

Q Wang, R Zennadi - Antioxidants, 2021 - mdpi.com
Sickle cell disease (SCD) is an inherited monogenic disorder and the most common severe
hemoglobinopathy in the world. SCD is characterized by a point mutation in the β-globin …

Inflammation in sickle cell disease

N Conran, JD Belcher - Clinical hemorheology and …, 2018 - content.iospress.com
The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant
pathophysiological consequences that result in hemolytic events and the induction of the …

Erythrocyte NADPH oxidase activity modulated by Rac GTPases, PKC, and plasma cytokines contributes to oxidative stress in sickle cell disease

A George, S Pushkaran… - Blood, The Journal …, 2013 - ashpublications.org
Chronic inflammation has emerged as an important pathogenic mechanism in sickle cell
disease (SCD). One component of this inflammatory response is oxidant stress mediated by …

[HTML][HTML] Red blood cells from endothelial nitric oxide synthase-deficient mice induce vascular dysfunction involving oxidative stress and endothelial arginase I

Z Zhuge, SMC Haworth, C Nihlén, LRRA Carvalho… - Redox Biology, 2023 - Elsevier
Background & aims Nitric oxide bioactivity (NO) from endothelial NO synthase (eNOS)
importantly contributes to the maintenance of vascular homeostasis, and reduced eNOS …

Consequences and management of iron overload in sickle cell disease

J Porter, M Garbowski - Hematology 2013, the American …, 2013 - ashpublications.org
The aims of this review are to highlight the mechanisms and consequences of iron
distribution that are most relevant to transfused sickle cell disease (SCD) patients and to …

Association between nitric oxide, oxidative stress, eryptosis, red blood cell microparticles, and vascular function in sickle cell anemia

E Nader, M Romana, N Guillot, R Fort… - Frontiers in …, 2020 - frontiersin.org
Chronic hemolysis, enhanced oxidative stress, and decreased nitric oxide (NO)
bioavailability promote vasculopathy in sickle cell anemia (SCA). Oxidative stress and NO …

L-glutamine for sickle cell disease: Knight or pawn?

A Sadaf, CT Quinn - Experimental Biology and Medicine, 2020 - journals.sagepub.com
Oxidative stress is an important contributor to the pathophysiology of sickle cell disease. The
pathways involved are complex and interlinked. L-glutamine is an amino acid with myriad …

Oxidative pathways in the sickle cell and beyond

AI Alayash - Blood Cells, Molecules, and Diseases, 2018 - Elsevier
Polymerization of deoxy sickle cell hemoglobin (HbS) is well recognized as the primary
event that triggers the classic cycles of sickling/unsickling of patients red blood cells (RBCs) …

[HTML][HTML] The evolving pharmacotherapeutic landscape for the treatment of sickle cell disease

SK Ballas - Mediterranean Journal of Hematology and Infectious …, 2020 - ncbi.nlm.nih.gov
Sickle cell disease (SCD) is an extremely heterogeneous disease that has been associated
with global morbidity and early mortality. More effective and inexpensive therapies are …