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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...

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Detalles Bibliográficos
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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: National Academy of Sciences 2020
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