INBDE (Integrated National Board Dental Examination) Practice Exam

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Sickle cell anemia is caused by the substitution of what amino acid for what?

  1. Phobic valine for polar glutamic acid

  2. Polar lysine for phobic valine

  3. Phobic leucine for polar aspartic acid

  4. Phobic valine for phobic leucine

The correct answer is: Phobic valine for polar glutamic acid

Sickle cell anemia is caused by a mutation in the gene encoding hemoglobin where a polar glutamic acid is replaced with a hydrophobic valine at the sixth position of the beta-globin chain. This substitution results in the formation of abnormal hemoglobin known as hemoglobin S. The presence of this abnormal hemoglobin distorts the shape of the red blood cells under low oxygen conditions, causing them to form a sickle shape and leading to various complications associated with sickle cell anemia. The other options are incorrect because they do not correspond to the specific amino acid substitution that causes sickle cell anemia.