Much of our understanding of human physiology, and of many aspects of pathology, has its
antecedents in laboratory and clinical studies of hemoglobin. Over the last century …

Haemoglobin (Hb) is widely known as the iron-containing protein in blood that is essential
for O 2 transport in mammals. Less widely recognised is that erythrocyte Hb belongs to a …

The β-thalassemia syndromes reflect deficient or absent β-globin synthesis usually owing to
a mutation in the β-globin locus. The relative excess of α-globin results in the formation of …

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 …

The pathophysiology of ineffective erythropoiesis in β-thalassemia is poorly understood. We
report that RAP-011, an activin receptor IIA (ActRIIA) ligand trap, improved ineffective …

The thalassemias, together with sickle cell anemia and its variants, are the world's most
common form of inherited anemia, and in economically undeveloped countries, they still …

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 …

The study of erythropoiesis aims to understand how red cells are formed from earlier
hematopoietic and erythroid progenitors. Specifically, the rate of red cell formation is …

In humans, β‐thalassemia dyserythropoiesis is characterized by expansion of early erythroid
precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective …

GAPDH, a heme chaperone, has been previously implicated in the incorporation of heme
into iNOS and soluble guanylyl cyclase (sGC). Since sGC is critical for myoglobin (Mb) heme …