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 …
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 …
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 …
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 …
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 …
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 …
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) …
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 …