Journal article
Targeting HbS Polymerization
Seminars in hematology, v 55(2)
Apr 2018
PMID: 30616807
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
The mutation of β6 from glu to val in hemoglobin is responsible for the polymer formation that leads to vaso-occlusion, and a range of severe consequences in sickle cell disease. The treatment of the disease can be addressed in many ways, but the prevention of polymer formation is one of the most fundamental approaches one can take. Such prevention includes affecting the polymer structure, or dilution of the fraction of polymerizable hemoglobin. The latter approach includes (1) induction of HbF, which does not itself, nor in hybrid form, join sickle polymers, or (2) restricting the allosteric change in hemoglobin that occurs in oxygen delivery, and which is required for polymer formation. These approaches will be critically reviewed, as well as the most recent developments that show the benefits of simply swelling the volume of the red cell.
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Details
- Title
- Targeting HbS Polymerization
- Creators
- Frank A. Ferrone - Drexel University
- Publication Details
- Seminars in hematology, v 55(2)
- Publisher
- Elsevier
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Physics
- Web of Science ID
- WOS:000438836900002
- Scopus ID
- 2-s2.0-85047196990
- Other Identifier
- 991019168807904721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Hematology