Explain the difference between normal hemoglobin protein and sickle cell disease protein?

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compassionate · Answer

Hi, Hemoglobin is the protein in red blood cells responsible for carrying oxygen from the lung to the rest of the body and for returning carbon dioxide from the rest of the body to the lung. Hemoglobin is comprised of multiple protein chains. Each of these chains contains a chemical group called “heme”. Each heme group can bind to an oxygen molecule. Sickle cell anemia is an autosomal recessive genetic disorder. A single amino acid substitution in hemoglobin is responsible for the development of sickle cell anemia. This single amino acid substitution results in the adherence of hemoglobin proteins to each other, forming long cables that can distort normal red blood cells into sickle shaped cells. Unlike normal red blood cells which are flexible and can fit through small capillaries, sickle red blood cells are inflexible and have the tendency to clog blood vessels. This can result in organ damage and severe pain in individuals with sickle cell anemia OpenStudy Ambassador: Compassionate. Source: http://star.mit.edu/media/uploads/biochem/exercises/version_2-0/hemoglobin-sickle_cell_exercise_ver8.pdf