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Article

Newer Aspects of the Pathophysiology of Sickle Cell Disease Vaso-Occlusion

1 Jan 2009

Abstract excerpt

Sickle cell disease is an inherited disorder of hemoglobin (Hb) synthesis, caused by a single nucleotide substitution (GTG>GAG) at the sixth codon of the beta-globin gene, leading to the production of a defective form of Hb, Hb S. When deoxygenated, Hb S polymerizes, damaging the sickle erythrocyte and it is this polymerization that is the primary indispensable event in the molecular pathogenesis of sickle cell...

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PMID: 19205968
DOI: 10.1080/03630260802625709

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