ABSTRACT

In his well-known paper of 1949 “Sickle Cell Anemia, a Molecular Disease” Linus Pauling and his collaborators at the California Institute of Technology described a new type of hemoglobin (Pauling et al., 1949). This new hemoglobin was characterized by its electrochemical behavior in the Tiselius apparatus. Under certain conditions the hemoglobin of sickle cell anemic patients migrated to one pole and the hemoglobin of non-sickling individuals to the other. Pauling’s team suggested that the different electrochemical behavior of sickle cell hemoglobin as compared to “normal” hemoglobin was due to a small structural change in the globin of the molecule. This alteration would allow the molecules under certain circumstances to stack onto one another, rendering the red blood cells rigid, to the point of bursting them. With this explanation Pauling interpreted the known clinical picture of sickle cell anemia as a “molecular disease”.