Sickle Cell Anemia, the First Molecular Disease: Overview of Molecular Etiology, Pathophysiology, and Therapeutic Approaches

The root cause of sickle cell disease is a single β-globin gene mutation coding for the sickle β-hemoglobin chain. Sickle hemoglobin tetramers polymerize when deoxygenated, damaging the sickle erythrocyte. A multifaceted pathophysiology, triggered by erythrocyte injury induced by the sickle hemoglob...

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Bibliographic Details
Main Author: Martin H. Steinberg
Format: Article
Language:English
Published: Hindawi Limited 2008-01-01
Series:The Scientific World Journal
Online Access:http://dx.doi.org/10.1100/tsw.2008.157