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sickle cell anaemia (Sickle cell (Mutation occurs in beta globin chain,…
sickle cell anaemia
Sickle cell
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if both genes carry Bglobin gene, HBSS
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point mutation of aa position 6, Valine not glutaric acid
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HbF left shift
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HbF higher affinity for O2 than HbA, so O2 dissociation curve shifts left
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cells last longer before irreversible sickling, buffer allows less low O2 tenison
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Sickling
O2 is given off at higher pO2 in sickle cell, giving O2 to organs & tissues in little demand
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Treatment
hydroxyurea can reduce risks of stroke in later life, new first base treatment