PATHOPHISIOLOGY OF SICKLE CELL DISEASE AND NEW DRUGS FOR THE TREATMENT

A homozygous mutation in the gene for b globin, a subunit of adult hemoglobin A (HbA), is the proximate cause of sickle cell disease (SCD). Sickle hemoglobin (HbS) shows peculiar biochemical properties, which lead to polymerizing when deoxygenated.  HbS polymerization is associated with a reduction...

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Bibliographic Details
Main Author: Lucia De Franceschi
Format: Article
Language:English
Published: PAGEPress Publications 2009-12-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Online Access:http://www.mjhid.org/index.php/mjhid/article/view/126