The hallmark of sickle cell disease is the polymerization of sickle haemoglobin due to a point
mutation in the β‐globin gene (HBB). Under low oxygen saturation, sickle haemoglobin …

Hematological data are listed in Table I. The mother, with a simple Hb Lufkin heterozygosity,
has a mild hemolytic anemia which is well-compensated and is comparable to that seen in …

One of the truly important structural problems of hemoglobins lies in the nature and location
of the intra-and intermolecular interactions that give rise to erythrocyte sickling, particularly in …

The sequential changes that occur during the precipitation on mild heating of the unstable
hemoglobins, Hb Christchurch, Hb Sydney, Hb Köln, and Hb A, were examined with …

We have used 13C/1H magnetic double-resonance spectroscopy to measure the amount of
sickle hemoglobin polymer within sickle erythrocytes as a function of oxygen saturation. We …

We have previously designed and investigated novel allosteric effectors of hemoglobin
(AEHs), as potential targeted treatment of sickle cell disease (SCD). In general, AEHs …

Materials and Methods Hemoglobin A and S Preparations. HbA was prepared as previously
described (Górecki et al., 1980) except final dialysis was done against 50 mM sodium …

Hb S variants containing Lys-β132→ Ala or Asn substitutions were engineered to evaluate
the consequences of the A helix destabilization in the polymerization process. Previous …

The replacement of a thymidine nucleotide for the normal adenine nucleotide in the sixth
codon of the p-globin gene (68) results in the substitution of valine for glutamic acid in the p …