[图书][B] Measurement of Kinetics of Sickle Hemoglobin Polymerization Using Absorbance: Implications for Therapy

EH Worth - 2023 - search.proquest.com
Therapeutic solutions to hinder the effects of sickle hemoglobin (HbS) polymerization are
highly sought after. Measuring the kinetics of polymerization of HbS using laser photolysis …

Molecular crowding limits the role of fetal hemoglobin in therapy for sickle cell disease

M Rotter, A Aprelev, K Adachi, FA Ferrone - Journal of molecular biology, 2005 - Elsevier
The dominant assumption central to most treatments for sickle cell anemia has been that
replacement of sickle hemoglobin (HbS) by fetal hemoglobin (HbF) would have major …

[PDF][PDF] Ligand Binding and Sickle Hemoglobin Polymerization Kinetics: Implication for Therapies

D Yosmanovich, M Rotter, A Aprelev, FA Ferrone - Biophysical Journal, 2009 - cell.com
Sickle Cell Disease results from a point mutation on the beta subgroups of hemoglobin.
When hemoglobin releases its four ligands it changes from a relaxed (R) structure to a tense …

Inhibiting the Polymerization of Sickle Hemoglobin: It's Complicated

DC Rees - The Hematologist, 2022 - ashpublications.org
Although hundreds of different hemoglobin variants have now been identified,
polymerization seems to be a unique property of HbS. 1 Polymerization has been …

Sparing effect of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S at physiologic ligand saturations.

WN Poillon, BC Kim, GP Rodgers… - Proceedings of the …, 1993 - National Acad Sciences
Recent interest in therapies for sickle cell anemia based on elevating fetal Hb has made
accurate estimates of the sparing effect of fetal Hb (Hb F) and other non-sickle Hbs on sickle …

Hemoglobin S Polymerization, Just the Beginning

FA Ferrone - Renaissance Of Sickle Cell Disease Research In …, 2007 - books.google.com
This chapter surveys the molecular events that cause individual hemoglobin tetramers to
become a stiff polymer mass with devastating consequences. It outlines the principles of …

[HTML][HTML] GBT021601, a next generation HbS polymerization inhibitor: results of safety, tolerability, pharmacokinetics and pharmacodynamics in adults living with sickle …

RCC Brown, A Redfern, E Lisbon, C Washington… - Blood, 2021 - Elsevier
Background: Sickle cell disease (SCD) is caused by polymerization of sickle hemoglobin
(HbS), resulting in red blood cell (RBC) sickling, RBC destruction, vaso-occlusion and end …

[PDF][PDF] Nucleation of sickle hemoglobin mixed with hemoglobin A: experimental and theoretical studies of hybrid-forming mixtures

M Rotter, D Yosmanovich, RW Briehl, S Kwong… - Biophysical journal, 2011 - cell.com
Sickle hemoglobin (HbS) is a point mutation of the two β subunits in normal Hb (HbA) that
leads to nucleated polymerization and accompanying pathology. We measured the rates of …

Kinetic assay shows that increasing red cell volume could be a treatment for sickle cell disease

Q Li, ER Henry, J Hofrichter, JF Smith… - Proceedings of the …, 2017 - National Acad Sciences
Although it has been known for more than 60 years that the cause of sickle cell disease is
polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment …

Solubility measurement of the sickle polymer

ME Fabry, SA Acharya, SM Suzuka… - … : Molecular Methods and …, 2003 - Springer
In sickle hemoglobin (HbS), a valine is substituted for glutamic acid in the sixth codon of the
globin chain. This change endows deoxyHbS, but not oxyHbS, with a new property: the …