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What is Which propey of hemoglobin is affected in sickle cell anemia? and explain in detail?
*Sickle cell disease is a hereditary hemoglobinopathy with structurally abnormal hemoglobinThe *Normal adult red cell contains 96% HbA (a2b2), 3% HbA2 (a2d2), and 1% fetal Hb (HbF, a2g2).*Sickle cell disease is caused by a point mutation in the 6th codon of b-globin chain*Occur primarily in African blacks (This high frequency stems from the protection afforded by HbS against falciparum malaria)*Heterozygotes for HbS is called as sickle cell trait (aabsb)*Homozygous for Hbs is called as sickle cell disease HbS (aabsbs) Pathogenesis:The solubility of hemoglobin is altered/ affected in HbS(Ref: Robbins 8/e p645, T. singh 1/e p270)