Blocking HbS Polymerization in SCD.
review · Level V
Where this comes from
- Record sourced from PubMed, PMID 32142671.
- Also identified by DOI 10.1016/j.cell.2020.01.019.
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Abstract
Sickle cell disease (SCD) is caused by a point mutation in the β-globin gene that creates hemoglobin S (HbS). Upon deoxygenation, HbS forms long polymers that distort the shape of red blood cells, causing hemolysis and vaso-occlusion. Voxelotor inhibits HbS polymerization, the root cause of SCD complications. To view this Bench to Bedside, open or download the PDF.
Medical subject headings
- Anemia, Sickle Cell
- Benzaldehydes
- Hemoglobin, Sickle
- Pyrazines
- Pyrazoles
- beta-Globins