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Pathophysiology of Sickle Cell Disease – Part 1 Video
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Video at a Glance
- Sickle cell disease is caused by hemoglobin deformation.
- A hemoglobin molecule is made up of four polypeptide chains that form a protein. The main type of hemoglobin is Hgb A, which has two alpha polypeptide chains and two beta chains.
- Sickle cell disease occurs when there’s a small mutation on the beta chain of Hgb A where glutamate is changed into valine.
- Hgb S forms a rigid polymer chain of Hgb S molecules, which produces long, rigid erythrocytes that tend to get stuck inside vessels, resulting in a vaso-occlusive crisis.
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