Sickle cell anemia, the first molecular disease: overview of molecular etiology, pathophysiology, and therapeutic approaches

MH Steinberg - The Scientific World Journal, 2008 - Wiley Online Library
The root cause of sickle cell disease is a single β‐globin gene mutation coding for the sickle
β‐hemoglobin chain. Sickle hemoglobin tetramers polymerize when deoxygenated …

Sickle cell disease vasculopathy: a state of nitric oxide resistance

KC Wood, LL Hsu, MT Gladwin - Free radical biology and medicine, 2008 - Elsevier
Sickle cell disease (SCD) is a hereditary hemoglobinopathy characterized by microvascular
vaso-occlusion with erythrocytes containing polymerized sickle (S) hemoglobin, erythrocyte …

Transient receptor potential vanilloid 1 mediates pain in mice with severe sickle cell disease

CA Hillery, PC Kerstein, D Vilceanu… - Blood, The Journal …, 2011 - ashpublications.org
Pain is the leading cause of emergency department visits, hospitalizations, and daily
suffering in individuals with sickle cell disease (SCD). The pathologic mechanisms leading …

A systematic review of known mechanisms of hydroxyurea-induced fetal hemoglobin for treatment of sickle cell disease

GD Pule, S Mowla, N Novitzky… - Expert review of …, 2015 - Taylor & Francis
Aim: To report on molecular mechanisms of fetal hemoglobin (HbF) induction by
hydroxyurea (HU) for the treatment of sickle cell disease. Study Design: Systematic review …

Red blood cell endothelial nitric oxide synthase: A major player in regulating cardiovascular health

A LoBue, SK Heuser, M Lindemann, J Li… - British Journal of …, 2023 - Wiley Online Library
Red blood cells (RBCs) have traditionally been seen as simple carriers of gases and
nutrients in the body. One important non‐canonical function of RBCs in the cardiovascular …

Allogeneic hematopoietic stem cell transplantation to cure sickle cell disease: a review

N Bhalla, A Bhargav, SK Yadav, AK Singh - Frontiers in Medicine, 2023 - frontiersin.org
Sickle cell disease (SCD) had first been mentioned in the literature a century ago.
Advancement in the molecular basis of the pathophysiology of the disease opens the door …

Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase …

E Nader, M Grau, R Fort, B Collins, G Cannas… - Nitric Oxide, 2018 - Elsevier
Hydroxyurea (HU) has been suggested to act as a nitric oxide (NO) donor in sickle cell
anemia (SCA). However, little is known about the HU NO-related effects on red blood cell …

[HTML][HTML] Identification of a soluble guanylate cyclase in RBCs: preserved activity in patients with coronary artery disease

MM Cortese-Krott, E Mergia, CM Kramer, W Lückstädt… - Redox Biology, 2018 - Elsevier
Endothelial dysfunction is associated with decreased NO bioavailability and impaired
activation of the NO receptor soluble guanylate cyclase (sGC) in the vasculature and in …

cGMP and cGMP-dependent protein kinase in platelets and blood cells

U Walter, S Gambaryan - cGMP: Generators, Effectors and Therapeutic …, 2009 - Springer
Platelets are specialized adhesive cells that play a key role in normal and pathological
hemostasis through their ability to rapidly adhere to subendothelial matrix proteins (platelet …

Pathophysiologically based drug treatment of sickle cell disease

MH Steinberg - Trends in Pharmacological Sciences, 2006 - cell.com
Sickle cell disease is a systemic disorder that is caused by a mutation (Glu6Val) in the gene
that encodes β globin. The sickle hemoglobin molecule (HbS) is a tetramer of two α-globin …