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Hemoglobin

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Hemoglobin Sickle cell anemia

Hemoglobin
• Hemoglobin is a protein that carries an oxygen from the lungs to the tissues. • Hemoglobin consists of 4 polypeptide subunits. Each of those subunits contains the heme group with an iron atom in its center. • This iron binds to the oxygen when it diffuses across the alveoli membranes in the lungs. So, each hemoglobin protein is able to bind to 4 oxygen molecules.(Wolfe, 2000)

Hemoglobin

Oxygenated and Deoxygenated Hemoglobin
• Oxygenated hemoglobin has oxygen attached to the heme group with an iron atom in its center. Oxygenated hemoglobin travels from the lungs to the tissues to deliver the oxygen. • Once it releases it’s oxygen, it then becomes deoxygenated and returns to the lungs. • The process of the oxygen binding is cooperative: the binding of 1 oxygen influences the binding of another. • As soon as a single heme group becomes oxygenated, the whole protein changes its shape. The hemes are dome-shaped. When oxygen binds, it pulls the iron atom down and the shape becomes flat. New shape makes it easier for the other 3 heme groups to become oxygenated. • It also causes a color change. Oxygenated hemoglobin is brightly red because of the attached oxygen. When hemoglobin becomes deoxygenated, it becomes the dark red color. (Wolfe, 2000)

Bohr effect
• pH is the amount of hydrogen ions in the solution. • Lower pH means a larger number of hydrogen ions and more acidic solution. • Higher pH means a lower number of hydrogen ions and more alkaline solution. • Hemoglobin acts as a buffer that helps to maintain a constant blood pH. A normal physiological pH is 7.4. • Hemoglobin is 100% saturated in the lungs. • When hemoglobin travels from the lungs it starts to release oxygen to the tissues. • Tissues at rest do not require a lot of oxygen. But during the exercise the pH of the blood decreases. The lower pH will cause hemoglobin to deliver more oxygen. • Bohr effect is a relationship between the pH and the ability of hemoglobin to release the oxygen. (Wolfe, 2000)

Bohr effect

Hemoglobin and myoglobin

The differences at the cellular level

Molecular differences

Sickle cell genetics
• If there is an amino acid substitution in one of the 4 chains of the hemoglobin molecule it causes a sickle cell anemia. • Sickle cell anemia is inherited, lifelong condition. • Sickle cell anemia is a recessive trait. Recessive traits require both parents to pass on the genes. If only one parent has the sickle cell anemia gene, the child who inherits it is a sickle cell carrier. Such person will have a sickle cell trait. (National Health Institutes, 2001)

Sickle cell genetics

• Ahern, K. (2012) Biochemistry free and easy. (version 1 ed.) E-book: Retrieved from http://biochem.science.oregonstate.edu/files/bbnew/imag es/BiochemistryFreeEasy1.pdf • Hudson-Miller, S. (n.d.) Oxy vs. Deoxy Hemoglobin. Retrieved from http://youtube/website. • Hudson-Miller, S. (n.d.) Sickle Cell Anemia. Retrieved from http://youtube/website • Hudson-Miller, S. (n.d.) The Bohr Effect. Hemoglobin and Myoglobin.Retrieved from http://youtube/website • Wolfe, G. (2000) Thinkwell biochemistry. Retrieved from http://wgu.thinkwell.com • National Institutes of Health. (n.d.) Retrieved from website: http://www.nhlbi.nih.gov/health/health-topics/topics/sca/

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