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Updated: Mar 24, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

Calibrating Sickle Cell Disease.

Donna Yosmanovich¹, Maria Rotter¹, Alexey Aprelev¹

¹Department of Physics, Drexel University, Philadelphia, PA 19104, USA.

Journal of Molecular Biology

|March 16, 2016

Summary

This summary is machine-generated.

Sickle cell disease (SCD) is a kinetic disorder. Ligand presence, like oxygen, inhibits HbS (hemoglobin S) sickling, offering insights for new SCD therapies.

Keywords:

kinetics ligands nucleation polymerization therapy

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Area of Science:

Hematology
Molecular Biology
Biophysics

Background:

Sickle cell disease (SCD) is characterized by mutated hemoglobin S (HbS) polymerizing and causing vaso-occlusion.
The kinetics of HbS sickling in the presence of physiological ligands are poorly understood.
Sickling is triggered by HbS losing ligands, leading to nucleation-dependent polymerization.

Purpose of the Study:

To investigate the effect of ligand presence on HbS nucleation rates.
To understand the role of quaternary structure in HbS sickling.
To develop predictive models for cell sickling fractions under varying ligand saturations.

Main Methods:

Measured nucleus formation rates in HbS with partial ligation of NO or CO.
Analyzed the influence of ligand binding on HbS quaternary structure (T-state).

Main Results:

Both NO and CO equivalently reduced HbS nucleation rates.
HbS nucleation requires the T (tense) quaternary structure.
Ligand presence inhibits nucleation even in the T-state conformation.

Conclusions:

Ligand binding significantly modulates HbS nucleation kinetics.
Understanding these kinetics is crucial for predicting sickling and designing therapies.
This research provides a basis for developing therapies that perturb oxygen affinity in SCD.