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Sickle Retinopathy

key information

source: Johns Hopkins Medicine

summary/abstract:

Sickle cell disease is caused by a small or point mutation in the hemoglobin molecule that is found in red blood cells (RBCs). Hemoglobin and the RBCs are important for carrying oxygen throughout the body in blood vessels. Because of the mutation, the hemoglobin makes polymers in the RBCs, which causes them to become rigid and abnormally shaped. Often RBCs appear sickle-shaped, giving the disease its name. These sickle RBCs (sRBCs) get stuck in blood vessels in retina and in many other organ systems, causing the tissue to become ischemic.

Dr. Lutty’s lab has developed a rat animal model for the occlusions caused by sRBCs in which human donor’s sRBCs are labeled with a fluorescent dye and injected into the blood of a rat. The number of cells that get stuck in retina can be counted at the end of the experiment, allowing therapies to prevent them from getting stuck to be evaluated.

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