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 …

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 …

[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 …

[PDF][PDF] A 50th order reaction predicted and observed for sickle hemoglobin nucleation

Z Cao, FA Ferrone - Journal of molecular biology, 1996 - academia.edu
The polymerization of sickle hemoglobin involves the assembly of 14 stranded fibers, which
are found in dense multibranched arrays (Briehl, 1995; White & Heagan, 1970) called …

[HTML][HTML] 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 …

Kinetics of sickle hemoglobin polymerization: II. A double nucleation mechanism

FA Ferrone, J Hofrichter, WA Eaton - Journal of molecular biology, 1985 - Elsevier
A double nucleation mechanism for the polymerization of sickle hemoglobin is described.
The mechanism accounts for all of the major kinetic observations: the appearance of a …

[PDF][PDF] Homogeneous nucleation in sickle hemoglobin: stochastic measurements with a parallel method

Z Cao, FA Ferrone - Biophysical journal, 1997 - cell.com
The homogeneous nucleation rate for sickle hemoglobin polymerization has been
measured for concentrations from 3.9 to 4.9 mM and temperatures from 13 degrees C to 35 …

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 …

Inhibition of Sickle Hemoglobin Polymerization as a Basis for Therapeutic Approaches to Sickle‐Cell Anemia

CT Noguchi, AN Schechter, JD Haley… - … Chemistry and Drug …, 2003 - Wiley Online Library
This chapter reviews current understanding of the molecular and cellular basis of the
pathophysiology of sickle‐cell anemia, with emphasis on the biophysics of the intracellular …

Kinetics of sickle hemoglobin polymerization: I. Studies using temperature-jump and laser photolysis techniques

FA Ferrone, J Hofrichter, WA Eaton - Journal of molecular biology, 1985 - Elsevier
Using a combination of laser photolysis and temperature-jump techniques, the kinetics of
hemoglobin S polymerization have been studied over a wide range of delay times (10− 3 to …