Voxelotor does not inhibit sickle hemoglobin fiber formation upon complete deoxygenation

EH Worth, MK Fugate, FA Ferrone - Biophysical Journal, 2023 - cell.com
The drug voxelotor (commercially known as Oxbryta) has been approved by the US Food
and Drug Administration for the treatment of sickle cell disease. It is known to reduce …

New developments in anti‐sickling agents: can drugs directly prevent the polymerization of sickle haemoglobin in vivo?

E Oder, MK Safo, O Abdulmalik… - British journal of …, 2016 - Wiley Online Library
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 …

Allosteric control of hemoglobin S fiber formation by oxygen and its relation to the pathophysiology of sickle cell disease

ER Henry, T Cellmer… - Proceedings of the …, 2020 - National Acad Sciences
The pathology of sickle cell disease is caused by polymerization of the abnormal
hemoglobin S upon deoxygenation in the tissues to form fibers in red cells, causing them to …

[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 …

Unit of Deoxygenated Sickle Hemoglobin Fibers

B Magdoff-Fairchild, CC Chiu - The Molecular Basis of Mutant …, 2013 - books.google.com
The importance of finding the structure of the deoxy-Hb S fiber resides in the information
about the intermolecular interactions that stabilize the fiber struc-ture. With this knowledge, it …

DESIGN, SYNTHESIS, BIOCHEMICAL AND TOXICOLOGICAL STUDIES OF POTENTIAL ANTI-SICKLING AGENTS.

ASA MEHANNA - 1988 - elibrary.ru
Sickle cell anemia is an inherited genetic disorder arising from a point mutation in the $\beta
$-globin gene that leads to the replacement of Glu by Val at the sixth position of the $\beta …

[引用][C] Covalent Chemical Modifiers of Sickle Cell Hemoglobina

H Ueno, Y Bai, JM Manning - Annals of the New York Academy …, 1989 - Wiley Online Library
In the past two decades, chemical modifiers of hemoglobin have contributed to our
understanding of the molecular disease-sickle cell anemia. These modifiers are of two types …

Treatment of sickle cell disease by increasing oxygen affinity of hemoglobin

ER Henry, B Metaferia, Q Li, J Harper… - Blood, The Journal …, 2021 - ashpublications.org
The issue of treating sickle cell disease with drugs that increase hemoglobin oxygen affinity
has come to the fore with the US Food and Drug Administration approval in 2019 of …

Covalent inhibitors of the gelation of sickle cell hemoglobin and their effects on function

JM Manning - Adv Enzymol Relat Areas Mol Biol, 1991 - books.google.com
Sickle cell anemia is a genetic disease in which the erythrocytes have lost their diskoid
shape (1) and their pliability because of the intracellular aggregates of hemoglobin S that …