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A novel mouse model of hemoglobin SC disease reveals mechanisms underlying beneficial effects of hydroxyurea

5 May 2025

Abstract excerpt

ABSTRACT: Sickle cell hemoglobin C (HbSC) disease results from compound heterozygosity of hemoglobin S (HbS) and hemoglobin C (HbC), comprising 30% of sickle cell disease (SCD). HbC induces red blood cell (RBC) dehydration/xerocytosis, which promotes sickling. HbSC-SCD causes significant morbidity despite being milder than homozygous HbSS-SCD. Current research/treatment strategies have focused on HbSS-SCD,...

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PMID: 40324066
DOI: 10.1182/blood.2024028136

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