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10.1016/j.mce.2013.09.003 141 WeishauptA.-K.RueckerL.MeinersT.SchwerdtleT.AvilaD

Abstract For over 100 years, clinicians and scientists have been unravelling the consequences of the A to T substitution in the -globin gene that produces haemoglobin S, which leads to the systemic manifestations of sickle cell disease (SCD), including vaso-occlusion, anaemia, haemolysis, organ injury and pain

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Given the progressive nature of the disorder, disease pathogenesis is mainly triggered by continual processes such as geometric accumulation of oxidative radicals brought about by cellular inclusions and mitochondrial dysfunction 8,9,10