From erythroblasts to mature red blood cells: organelle clearance in mammals

M Moras, SD Lefevre, MA Ostuni - Frontiers in physiology, 2017 - frontiersin.org
Erythropoiesis occurs mostly in bone marrow and ends in blood stream. Mature red blood
cells are generated from multipotent hematopoietic stem cells, through a complex maturation …

Red blood cell metabolism in vivo and in vitro

A D'Alessandro, AT Anastasiadi, VL Tzounakas… - Metabolites, 2023 - mdpi.com
Red blood cells (RBC) are the most abundant cell in the human body, with a central role in
oxygen transport and its delivery to tissues. However, omics technologies recently revealed …

Oxidative stress in healthy and pathological red blood cells

F Orrico, S Laurance, AC Lopez, SD Lefevre… - Biomolecules, 2023 - mdpi.com
Red cell diseases encompass a group of inherited or acquired erythrocyte disorders that
affect the structure, function, or production of red blood cells (RBCs). These disorders can …

Therapeutic strategies for sickle cell disease: towards a multi-agent approach

MJ Telen, P Malik, GM Vercellotti - Nature reviews Drug discovery, 2019 - nature.com
For over 100 years, clinicians and scientists have been unravelling the consequences of the
A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the …

Circulating mitochondrial DNA is a proinflammatory DAMP in sickle cell disease

L Tumburu, S Ghosh-Choudhary… - Blood, The Journal …, 2021 - ashpublications.org
The pathophysiology of sickle cell disease (SCD) is driven by chronic inflammation fueled by
damage associated molecular patterns (DAMPs). We show that elevated cell-free DNA …

The worst things in life are free: the role of free heme in sickle cell disease

OT Gbotosho, MG Kapetanaki, GJ Kato - Frontiers in immunology, 2021 - frontiersin.org
Hemolysis is a pathological feature of several diseases of diverse etiology such as
hereditary anemias, malaria, and sepsis. A major complication of hemolysis involves the …

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 …

Retention of functional mitochondria in mature red blood cells from patients with sickle cell disease

C Moriconi, M Dzieciatkowska, M Roy… - British journal of …, 2022 - Wiley Online Library
Sickle cell disease (SCD) is an inherited blood disorder characterized by sickled red blood
cells (RBCs), which are more sensitive to haemolysis and can contribute to disease …

Oral ferroportin inhibitor ameliorates ineffective erythropoiesis in a model of β-thalassemia

V Manolova, N Nyffenegger, A Flace… - The Journal of …, 2020 - Am Soc Clin Investig
β-Thalassemia is a genetic anemia caused by partial or complete loss of β-globin synthesis,
leading to ineffective erythropoiesis and RBCs with a short life span. Currently, there is no …

Targeting novel mechanisms of pain in sickle cell disease

H Tran, M Gupta, K Gupta - Blood, The Journal of the American …, 2017 - ashpublications.org
Patients with sickle cell disease (SCD) suffer from intense pain that can start during infancy
and increase in severity throughout life, leading to hospitalization and poor quality of life. A …