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Le malattie molecolari


Note:

Ex Molecular desease
Bill Castle, a physician from Peter Bent Brigham Hospital and Children's Hospital in Boston, talked about sickle cell anemia. I was not especially interested in what he was saying, because at that time I felt that diseases were just too complicated for me to attempt to understand. He mentioned that the red blood cells were twisted out of shape in the blood of patients with this disease. I thought to myself, there are probably thousands of different chemical substances in the red blood cell, and I don't think I can understand why a cell would be twisted out of shape. Then, went on to explain that they twisted out of shape in venous blood, but resume their normal flattened spherical shape in arterial blood. Within a few seconds the idea flashed through my mind that red cells contain a great amount of hemoglobin - about one-seventh of the content of a cell is hemoglobin molecules - and that difference between venous blood and arterial blood is that in venous blood the hemoglobin is present as hemoglobin itself, whereas in arterial blood it becomes oxyhemoglobin because there are oxygen molecules attached to the iron atoms. .... It immediately occurred to me that sickle-cell anemia must be a disease of the hemoglobin molecule, with sickle-cell hemoglobin having an abnormal structure.

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This was in March 1945. The deduction was a hypothesis that needed to be tested. Pauling immediately began assembling a group of research workers who could attack the problem. Harvey Itano, with a B.S. in Chemistry from the University of California and an M.D. from St. Louis University, joined Pauling's group to work on a Ph.D. Jon singer, a postdoctoral fellow, helped out. Work proceeded slowly. The article describing their work appeared in late November 1949, almost five years after Pauling's original insight.