[PDF][PDF] GBT440 increases haemoglobin oxygen affinity, reduces sickling and prolongs RBC half-life in a murine model of sickle cell disease
FA Ferrone - Br J Haematol, 2016 - academia.edu
In their paper in the current issue of the British Journal of Haematology, Oksenberg et al
(2016) have taken a substantial step toward providing a second drug to treat the symptoms …
(2016) have taken a substantial step toward providing a second drug to treat the symptoms …
A phase 3 randomized trial of voxelotor in sickle cell disease
E Vichinsky, CC Hoppe, KI Ataga… - … England Journal of …, 2019 - Mass Medical Soc
Abstract Background Deoxygenated sickle hemoglobin (HbS) polymerization drives the
pathophysiology of sickle cell disease. Therefore, direct inhibition of HbS polymerization has …
pathophysiology of sickle cell disease. Therefore, direct inhibition of HbS polymerization has …
Mapping polymerization and allostery of hemoglobin s using point mutations
P Weinkam, A Sali - The Journal of Physical Chemistry B, 2013 - ACS Publications
Hemoglobin is a complex system that undergoes conformational changes in response to
oxygen, allosteric effectors, mutations, and environmental changes. Here, we study allostery …
oxygen, allosteric effectors, mutations, and environmental changes. Here, we study allostery …
Hemoglobin polymerization in sickle cells studied by circular polarized light scattering
We have studied intracellular polymerization of hemoglobin S in suspensions of small
populations of sickle cells using circular polarized light scattering. We argue that the …
populations of sickle cells using circular polarized light scattering. We argue that the …
Intracellular polymerization of sickle hemoglobin: disease severity and therapeutic goals.
CT Noguchi, GP Rodgers… - Progress in Clinical and …, 1987 - europepmc.org
We have demonstrated that the extent of intracellular polymerization of deoxyhemoglobin S
can be predicted from knowledge of intracellular hemoglobin concentration, composition …
can be predicted from knowledge of intracellular hemoglobin concentration, composition …
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 …
increasing haemoglobin S (Hb S) oxygen affinity, two liganded derivatives of Hb S have …
Band 3 catalyzes sickle hemoglobin polymerization
We have measured homogeneous and heterogeneous nucleation rates of sickle
hemoglobin (HbS) in the presence of a strongly binding deletion mutant of the cytoplasmic …
hemoglobin (HbS) in the presence of a strongly binding deletion mutant of the cytoplasmic …
Systematic enhancement of polymerization of recombinant sickle hemoglobin mutants: Implications for transgenic mouse model for sickle cell anemia
To provide quantitative information on the sites that promote polymerization of sickle
hemoglobin (HbS) after formation of the initial hydrophobic bond involving Val-6 (β)[E6V (β)] …
hemoglobin (HbS) after formation of the initial hydrophobic bond involving Val-6 (β)[E6V (β)] …
Thermodynamic mechanism of free heme action on sickle cell hemoglobin polymerization
A Aich, W Pan, PG Vekilov - AIChE Journal, 2015 - Wiley Online Library
For insights into the mechanisms of heme action on the rate of sickle cell hemoglobin
polymerization, we determine the erythrocytic concentration of free heme using a novel …
polymerization, we determine the erythrocytic concentration of free heme using a novel …