[引用][C] Hemoglobin S gelation and sickle cell disease

WA Eaton, J Hofrichter - 1987

[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 …

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 …

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 …

Hemoglobin polymerization in sickle cells studied by circular polarized light scattering

CT Gross, H Salamon, AJ Hunt, RI Macey… - … et Biophysica Acta (BBA …, 1991 - Elsevier
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 …

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 …

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 …

Band 3 catalyzes sickle hemoglobin polymerization

MA Rotter, H Chu, PS Low, FA Ferrone - Biophysical chemistry, 2010 - Elsevier
We have measured homogeneous and heterogeneous nucleation rates of sickle
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

X Li, UA Mirza, BT Chait… - Blood, The Journal of the …, 1997 - ashpublications.org
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)] …

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 …