Understanding sickle cell disease, its role in oxygen transportation and its genetic cure from the dynamics of haemoglobin
Sickle cell disease is a group of blood disorders, the most common is the sickle cell anemia. It is a genetic mutation of the oxygen- carrying protein hemoglobin found in red blood cells. This leads to a rigid, sickle-like shape. This hemoglobin (HbS) is a variant of normal human hemoglobin (HbA). From a molecular point of view, the mutation induces polymerization of HbS, when the protein is in its deoxygenated state and very concentrated. Recently, the Henri-Mondor hospital developed a genetic cure for sickle cell anemia and successfully cured a first patient. After 15 months, a mixture of HbS and HbA composes the total amount of patient hemoglobin and no sickle cell anemia crises are observed. In this experiment, we want to investigate the dynamics of Hb using NSE. HbS and of the mixture of HbS and therapeutic HbA will be investigated to characterise the anomalous diffusion of HbS induced by its polymerisation and understand how the therapeutic hemoglobin affects the diffusion process. This is a crucial step in the understanding of the genetic cure. Different oxygen partial pressure will be investigated to simulate different body environment (lungs, heart, tissues).
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MARIANI Giacomo; BARTOLUCCI Pablo; BARTOLUCCI Pablo; CHENNEVIERE Alexis; CZAKKEL Orsolya; FARAGO Bela; HEBERT Nicolas; KIGER Laurent; LONGEVILLE Stephane and PHAM HUNG D'ALEXANDRY D'ORENGI. (2021). Understanding sickle cell disease, its role in oxygen transportation and its genetic cure from the dynamics of haemoglobin. Institut Laue-Langevin (ILL) doi:10.5291/ILL-DATA.8-04-858
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