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Allosteric control of hemoglobin S fiber formation by oxygen and its relation to the pathophysiology of sickle cell disease
The pathology of sickle cell disease is caused by polymerization of the abnormal hemoglobin S upon deoxygenation in the tissues to form fibers in red cells, causing them to deform and occlude the circulation. Drugs that allosterically shift the quaternary equilibrium from the polymerizing T quaterna...
Autores principales: | Henry, Eric R., Cellmer, Troy, Dunkelberger, Emily B., Metaferia, Belhu, Hofrichter, James, Li, Quan, Ostrowski, David, Ghirlando, Rodolfo, Louis, John M., Moutereau, Stéphane, Galactéros, Frédéric, Thein, Swee Lay, Bartolucci, Pablo, Eaton, William A. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
National Academy of Sciences
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7334536/ https://www.ncbi.nlm.nih.gov/pubmed/32527859 http://dx.doi.org/10.1073/pnas.1922004117 |
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