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Commentary on and reprint of Pauling L, Itano HA, Singer SJ, Wells IC, Sickle cell anemia, a molecular disease, in Science (1949) 110:543–548

2000; Maney Publishing; Linguagem: Inglês

10.1016/b978-012448510-5/50128-x

1607-8454

Marilyn Lichtman, Jeffrey M. Spivak, L BOXER, Sanford J. Shattil, E HENDERSON,

Iron Metabolism and Disorders

Publisher SummaryThe erythrocytes of certain individuals possess the capacity to undergo reversible changes in shape, in response to changes in the partial pressure of oxygen. The main observable difference between the erythrocytes of sickle cell trait and sickle cell anemia has been that, a considerably greater reduction in the partial pressure of oxygen is required for a major fraction of the trait cells to sickle than for the anemia cells. The evidence available at the time that the investigation has begun indicated that the process of sickling might be intimately associated with the state and the nature of the hemoglobin within the erythrocyte. The hemoglobin within the sickled cells appears to aggregate into one or more foci and the cell membranes collapse. According to titration data obtained by us, the acid-base titration curve of normal human carbonmonoxyhemoglobin is nearly linear in the neighborhood of the isoelectric point of the protein, and a change of one pH unit in the hemoglobin solution in this region is associated with a change in net charge on the hemoglobin molecule of about 13 charges per molecule.