Sickle‐cell haemoglobin polymerization: is it the primary pathogenic event of sickle‐cell anaemia?

PG Vekilov - British journal of haematology, 2007 - Wiley Online Library
Sickle cell anaemia is associated with a mutant haemoglobin, HbS, which forms polymers in
the red blood cells of patients. The primary role of the HbS polymerization for the …

Free heme and the polymerization of sickle cell hemoglobin

VV Uzunova, W Pan, O Galkin, PG Vekilov - Biophysical journal, 2010 - cell.com
In search of novel control parameters for the polymerization of sickle cell hemoglobin (HbS),
the primary pathogenic event of sickle cell anemia, we explore the role of free heme, which …

Two-step mechanism of homogeneous nucleation of sickle cell hemoglobin polymers

O Galkin, W Pan, L Filobelo, RE Hirsch, RL Nagel… - Biophysical journal, 2007 - cell.com
Sickle cell anemia is a debilitating genetic disease that affects hundreds of thousands of
babies born each year worldwide. Its primary pathogenic event is the polymerization of a …

Mechanisms of homogeneous nucleation of polymers of sickle cell anemia hemoglobin in deoxy state

O Galkin, PG Vekilov - Journal of molecular biology, 2004 - Elsevier
The primary pathogenic event of sickle cell anemia is the polymerization of the mutant
hemoglobin (Hb) S within the red blood cells, occurring when HbS is in deoxy state in the …

Nucleation, fiber growth and melting, and domain formation and structure in sickle cell hemoglobin gels

RW Briehl - Journal of molecular biology, 1995 - Elsevier
Pathogenesis in sickle cell disease depends on polymerization and gelation of
deoxyhemoglobin S. Under the double nucleation model, polymerization is initiated by …

[HTML][HTML] The intracellular polymerization of sickle hemoglobin and its relevance to sickle cell disease

CT Noguchi, AN Schechter - Blood, 1981 - Elsevier
In the last few years, the understanding of the molecular basis of sickle cell disease has
progressed rapidly. It is now possible to describe the structure of the gel of polymerized …

The kinetics of nucleation and growth of sickle cell hemoglobin fibers

O Galkin, RL Nagel, PG Vekilov - Journal of molecular biology, 2007 - Elsevier
Polymerization of sickle cell hemoglobin (HbS) in deoxy state is one of the basic events in
the pathophysiology of sickle cell anemia. For insight into the polymerization process, we …

Intracellular hemoglobin S polymerization and the clinical severity of sickle cell anemia

WN Poillon, BC Kim, O Castro - Blood, The Journal of the …, 1998 - ashpublications.org
Recent work has enabled us to quantitate the four variables (2, 3-DPG concentration, pHi,
non-S hemoglobin composition, and O2 saturation) that modulate the equilibrium solubility …

Hemoglobin S polymerization and sickle cell disease: A retrospective on the occasion of the 70th anniversary of Pauling's Science paper

WA Eaton - American journal of hematology, 2020 - Wiley Online Library
Abstract 70 years ago, Linus Pauling, the legendary genius of 20th century chemistry,
published his famous work on the molecular cause of sickle cell disease, a paper that gave …

Establised and experimental treatments for sickle cell disease

L De Franceschi, R Corrocher - haematologica, 2004 - iris.univr.it
Sickle cell disease (SCD) is characterized by the presence of sickle hemoglobin (HbS),
which has the unique property of polymerizing when deoxygenated. The sickling process is …