Sickle-cell anemia hemoglobin

If hydrophobic interactions are involved in this association, as has been suggested by Eauzmann (1959) on the basis of this observation, it should be sensitive to the introduction of small amounts of the appropriate weakly protic nonaqueous solvents. The endothermic association of sickle cell anemia hemoglobin (Murayama, 1956) is another case to investigate. 

Ingram VM. A specific chemical difference between globins of normal and sickle-cell anemia hemoglobins. Nature 1956 178 792-4. 

Hemoglobin Human Sickle cell anemia Hemoglobin S 180, 275-278... 

Our blood contains the complex protein hemoglobin, which carries oxygen from the lungs to other parts of the body. In the genetic disease known as sickle-cell anemia, hemoglobin molecules are abnormal and have a lower solubility, especially in their unoxygenated form. Consequently, as much as 85% of the hemoglobin in red blood cells crystallizes out of solution. 

The isoelectric points are listed in Table 1. These results prove that the electrophoretic difference between normal hemoglobin and sickle cell anemia hemoglobin... 

The fact that the blood of individuals with sickle-cell trait usually contains normal hemoglobin in somewhat larger amount than sickle-cell-anemia hemoglobin, the ratio of the two being somewhat different for different individuals, has been ascribed recently by Itano to a genetic difference of rate of manufacture of normal hemoglobin, as compared with the rate of manufacture of sickle-cell-anemia hemoglobin. 

Fig. 3. The electrophoretic patterns for (o) normal adult human hemoglobin, (fe) sickle-cell-anemia hemoglobin, from the red cells of patients with sicklecell anemia, (c) sickle-cell-trait hemoglobin, which is indicated to be a mixture of normal adult human hemoglobin and sickle-cell-anemia hemoglobin, and (

Havinga E., and H. A. Itano, Electrophoretic studies on the globins of sickle-cell-anemia hemoglobin and normal adult human hemoglobin unpublished investigation. 

Fig. 5. At the left, molecules of normal hemoglobin or of oxygenated sickle-cell-anemia hemoglobin are shown, with random orientations, and at about the average distance apart characteristic of red-cell contents. At the right long strings of molecules of deoxygenated sickle-cell-anemia hemoglobin are shown, assuming the parallel orientation characteristic of the nematic liquid crystals that presumably form within the red cells in the venous blood of patients with sickle-cell anemia, and twist the red cells into the abnormal shape characteristic of the disease.

The Properties of Sickle-Cell-Anemia Hemoglobin AND THE Mechanism of Sickling... 

Sickle-cell-anemia hemoglobin is closely similar to normal adult human hemoglobin in most of its properties. The two proteins have approximately the same sedimentation and diffusion constants,... 

Fig. 2. Hemoglobin formed in stroma-free solutions of deoxygenated sickle-cell-anemia hemoglobin. Phase photomicrography, X 375. From John W. Harris, Proc. Soc. Exptl. Biol, and Med. 75, 197 (1950).

---