Question

Sickle cell anemia is a genetic disease resulting from a single amino acid substitution (glutamate to valine in the sixth position, or E6V) in the hemoglobin β chain. This mutation causes hemoglobin to polymerize into strands in the deoxygenated state, leading to the erythrocytes forming a sickled shape. Speculate as to why the mutation leads to the polymerization of hemoglobin and why this only happens in the deoxygenated state.

Answer 1

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Answer: The defect to raline is caused by the Substitution of Glutamate the sixth position of ß- globin chain. in Hbs C 6 Glu vall > - G.GIU P2) - Normal Abnormal in (GLUT > Hydrophilic) (VALT) Hychopnobizi! * This residue its Present on the outer surface of the Hemoglobin molecule. lligade in * This change produce a Sticky hydrophobie spot on the n surface of molecule This Make deo xu the less soluble than deoxy HbA & than Insoluble aggregates than deoxy Hbs forms polymer agg into tubular fibers tatus Hydrophobic studie Deoxy Hbs En Releasing On very we well but Hb rapidaly * This make deo xyHbs less in tranformed in crystalline form known as Defective Hb t Tac toids test mw tigutuna) After releasing on This have sticky ends and make interaction with othe hydrophobic ß- Chains of other Hemoglobin. when ultimately Sticky ends. heads to sickling of cell. m203 Abs polyon mobs HOA 1 Defective RBC Normal RBC

I sickling V - # The deoxygenating form causing 02 – No Sickie NO O2 -> Sickie rol e ole * This is because Defecting Has transporting oxygen , Very well but after releasing oxygen it become corte - have sticky ends and make polymess with other a rather than accepting oxygenom allars * This polymerization leads to the sickled RBC * Repeating sicking Leads to the membiane damage of RBC, which is leads to hemolysis offee and Ultimately destroy RBC", waliyopis sloween I NO O2 Sickle membrane Hemolysis B ow RBC in vay Damage witte To *if we provide on Hbs work normally but in deoxy. Conditing it will stast Clumping