Alpha
Beta
Beta
Alpha
Oxygen
Iron Atoms
Heme Groups
Oxygenated Hemoglobin * Formed via transportation of O2 to cells in tissue * O2 adheres to heme protein in Hgb * T (taut state) R (relaxed state) makes binding easier or releases De-oxygenated Hemoglobin * is not bound to oxygen molecule * Higher absorption * Blue-ish in color
Bohr’s Effect
CO2 produced through citric acid cycle, Hg carries oxygen from lungs to body's tissues. Hg releases oxygen for CO2 and affects pH levels. Normal pH range is 7.2 - 7.4
↓ pH causes ↑ in the amount of oxygen being released in hemoglobin. An elevated pH will cause the oxygen to bind the hemoglobin proteins in RBCs.
("Hemoglobin," n.d.)
RBCs are round cells that have concaved centers. They are flexible making it easy to move through blood vessels. Sickle cell RBC's are developed from mutations in DNA - mRNA transcriptions. They are crescent shape and become fibrous. This causes them to stick to one another. Once they begin to stick, the deoxygenated cells are unable to travel to the lungs to receive oxygen. As the CO2 builds up this causes lack of oxygen to the tissues causing pain. Sickle Cell cells also cause anemia due to the fact cells die faster than normal RBCs.
Sickle cell disease is an inherited autosomal recessive pattern disorder. For a child to inherit the disease both parents must have the trait and pass down a defective copy of the gene. Those who inherit one normal hemoglobin copy gene and one mutated copy carry the trait. Although normal, they are still able pass the mutant defective gene to their children.
Children of 2 carrier parents have a 25% chance of having normal hemoglobin, a 50% chance of inheriting the trait, and a 25% chance of inheriting Sickle Cell.
References
Retrieved from http://learn.genetics.utah.edu/content/disorders/singlegene/sicklecell/images/sicklecell.jpg
Hemoglobin. (n.d.). Retrieved from http://www.bio.davidson.edu/Courses/Molbio/MolStudents/spring2003/Stonestreet/Hemoglobinpage.htm
What Causes Sickle Cell Anemia? - NHLBI, NIH. (n.d.). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/sca/causes