Article
Pathophysiology and therapy for haemoglobinopathies. Part I: sickle cell disease.
Expert reviews in molecular medicine - 28 Apr 2006
Madigan Catherine, Malik Punam
Abstract excerpt
In sickle cell disease, a single base pair substitution in the gene encoding the beta-globin chain of the haemoglobin molecule gives rise to a surprisingly broad spectrum of pathophysiological and clinical manifestations. Inflammation, endothelial activation, red blood cell membrane abnormalities and altered availability of vasoactive factors characterise this disorder. Clinically, patients suffer from a host of...
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