Open AccessBlocking HbS Polymerization in SCD
Author(s) -
Guillaume Lettre
Publication year - 2020
Publication title -
cell
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 26.304
H-Index - 776
eISSN - 1097-4172
pISSN - 0092-8674
DOI - 10.1016/j.cell.2020.01.019
Subject(s) - hemolysis , biology , polymerization , deoxygenation , point mutation , hemoglobin , mutation , gene , immunology , microbiology and biotechnology , biochemistry , polymer , catalysis , chemistry , organic chemistry
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.
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