[HTML][HTML] Oxidative stress in β-thalassaemia and sickle cell disease

S Voskou, M Aslan, P Fanis, M Phylactides… - Redox biology, 2015 - Elsevier
Sickle cell disease and β-thalassaemia are inherited haemoglobinopathies resulting in
structural and quantitative changes in the β-globin chain. These changes lead to instability …

Emerging EPO and EPO receptor regulators and signal transducers

D Kuhrt, DM Wojchowski - Blood, The Journal of the American …, 2015 - ashpublications.org
As essential mediators of red cell production, erythropoietin (EPO) and its cell surface
receptor (EPO receptor [EPOR]) have been intensely studied. Early investigations defined …

Physiology and pathophysiology of iron in hemoglobin-associated diseases

TD Coates - Free Radical Biology and Medicine, 2014 - Elsevier
Iron overload and iron toxicity, whether because of increased absorption or iron loading from
repeated transfusions, can be major causes of morbidity and mortality in a number of chronic …

The pyruvate kinase activator mitapivat reduces hemolysis and improves anemia in a β-thalassemia mouse model

A Matte, E Federti, C Kung, PA Kosinski… - The Journal of …, 2021 - Am Soc Clin Investig
Anemia in β-thalassemia is related to ineffective erythropoiesis and reduced red cell
survival. Excess free heme and accumulation of unpaired α-globin chains impose …

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 …

Band 3 protein function and oxidative stress in erythrocytes

A Remigante, R Morabito… - Journal of cellular …, 2021 - Wiley Online Library
Abstract Band 3 protein (B3p), anion transporter, allows the HCO3−/Cl− exchange across
plasma membrane and plays an important role for erythrocytes homeostasis. In addition …

Pharmacological induction of fetal hemoglobin in β-thalassemia and sickle cell disease: An updated perspective

R Bou-Fakhredin, L De Franceschi, I Motta… - Pharmaceuticals, 2022 - mdpi.com
A significant amount of attention has recently been devoted to the mechanisms involved in
hemoglobin (Hb) switching, as it has previously been established that the induction of fetal …

Erythrocytes as a preferential target of oxidative stress in blood

J Fujii, T Homma, S Kobayashi, P Warang… - Free Radical …, 2021 - Taylor & Francis
Red blood cells (RBC) are specifically differentiated to transport oxygen and carbon dioxide
in the blood and they lack most organelles, including mitochondria. The autoxidation of …

d‐Galactose induced early aging in human erythrocytes: Role of band 3 protein

A Remigante, S Spinelli, V Trichilo… - Journal of cellular …, 2022 - Wiley Online Library
Aging, a time‐dependent multifaceted process, affects both cell structure and function and
involves oxidative stress as well as glycation. The present investigation focuses on the role …

Ineffective erythropoiesis in β-thalassaemia: key steps and therapeutic options by drugs

F Longo, A Piolatto, GB Ferrero, A Piga - International Journal of …, 2021 - mdpi.com
β-thalassaemia is a rare genetic condition caused by mutations in the β-globin gene that
result in severe iron-loading anaemia, maintained by a detrimental state of ineffective …