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) …

Sickle cell anaemia (SCA) is the consequence of abnormal haemoglobin production due to
an inherited point mutation in the β‐globin gene. The resulting haemoglobin tetramer is …

Heme is an essential molecule in aerobic organisms. Heme consists of protoporphyrin IX
and a ferrous (Fe2+) iron atom, which has high affinity for oxygen (O2). Hemoglobin, the …

Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin
gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS) …

The contribution of intracellular hemoglobin (Hb) oxidation to RBC-derived microparticle
(MP) formation is poorly defined in sickle cell disease (SCD). Here we report that sickle Hb …

Erythrocytes have an environment of continuous pro-oxidant generation due to the presence
of hemoglobin (Hb), which represents an additional and quantitatively significant source of …

Sickle cell disease is an inherited disorder of hemoglobin (Hb) synthesis, caused by a single
nucleotide substitution (GTG> GAG) at the sixth codon of the β-globin gene, leading to the …

Sickle cell disease (SCD) is a monogenetic disorder marked by hemolytic anemia and vaso-
occlusive complications. The hallmark of SCD is the intracellular polymerization of sickle …

A homozygous mutation in the gene for β globin, a subunit of adult hemoglobin A (HbA), is
the proximate cause of sickle cell disease (SCD). Sickle hemoglobin (HbS) shows peculiar …

The first coherent pathophysiological scheme for sickle cell disease (SCD) emerged in the
sixties-seventies based on an extremely detailed description of the molecular mechanism by …