Increasing haemoglobin oxygen affinity to prevent sickling: abnormal haemoglobin variants as models

IM Franklin, ER Huehns… - British journal of …, 1986 - Wiley Online Library
TWO abnormal high oxygen affinity haemoglobins have been studied for their ability to
inhibit the polymerization of deoxy‐Hb S. They were used as models to predict the effect of …

Sickle cell disease: the proportion of liganded haemoglobin needed to prevent crises

IM Franklin, MA Rosemeyer… - British Journal of …, 1983 - Wiley Online Library
In an attempt to predict the likelihood of successfully treating sickle cell disease by
increasing haemoglobin S (Hb S) oxygen affinity, two liganded derivatives of Hb S have …

[引用][C] The effect of β73Asn on the interactions of sickling hemoglobins

RM Bookchin, RL Nagel, HM Ranney - Biochimica et Biophysica Acta (BBA …, 1970 - Elsevier
There is considerable evidence that deoxyhemoglobin S molecules tend to aggregate with
formation of polymers, but the nature of the binding sites between adjacent hemoglobin …

The concentration dependence of the oxygen affinity of haemoglobin S

A May, ER Huehns - British journal of haematology, 1975 - Wiley Online Library
The effect of the concentration of haemoglobin S (Hb S) on its oxygen‐dissociation
properties was studied using either reconstituted Hb‐S cells of different mean corpuscular …

Haemoglobin α2β223Val → Ile produced in Escherichia coli facilitates Hb S polymerization

J Pagnier, V Baudin‐Chich, N Lacaze… - British journal of …, 1990 - Wiley Online Library
The doubly substituted variant Hb S‐Antilles (β6 Glu→ Val, β3 Val→ Ile) produces sickling in
heterozygous carriers. The Csat value for pure deoxyHb S‐Antilles is nearly half that of …

Oxygen affinity as an index of hemoglobin S polymerization: a new micromethod

RE Benesch, R Edalji, S Kwong, R Benesch - Analytical biochemistry, 1978 - Elsevier
The decrease in oxygen affinity with increasing hemoglobin concentration, which occurs in
solutions of pure hemoglobin S, can be used to determine the minimum concentration at …

Covalent inhibitors of sickling

WC Mentzer Jr, R Pennathur-Das… - The Molecular Basis …, 1981 - books.google.com
The effectiveness of cyanate as an in vitro inhibitor of sickling'and the preliminary evidence
of its clinical usefulness when administered via an extracorporeal route” have stimulated the …

[HTML][HTML] Novel structurally-modified allosteric effectors of hemoglobin exhibit superior antisickling properties

O Abdulmalik, T Deshpande, M Ghatge, Y Zhang… - Blood, 2014 - Elsevier
Sickle cell disease (SCD) continues to cause significant morbidity, mortality and healthcare
disparities. Despite considerable progress in understanding the underlying pathophysiology …

Inhibition of sickling after reduction of intracellular hemoglobin concentration with an osmotic pulse: characterization of the density and hemoglobin concentration …

RS Franco, R Barker-Gear, R Green - Blood cells, 1993 - europepmc.org
Hemoglobin S polymerization is markedly dependent on intracellular hemoglobin
concentration. In the studies presented here, sickle RBC were subjected to a transient …

Effect of the beta 73 amino acid on the hydrophobicity, solubility, and the kinetics of polymerization of deoxyhemoglobin S.

K Adachi, J Kim, TR Kinney, T Asakura - Journal of Biological Chemistry, 1987 - ASBMB
The role of Asp-beta 73 on the surface hydrophobicity and solubility of hemoglobin was
studied using Hb A, Hb S, Hb C Harlem (alpha 2 beta 2Val-6, Asn-73), and Hb Korle Bu …