Polyamines and membrane proteins in sickle cell disease.
The polyamines, putrescine, spermidine, and spermine have been implicated in cellular growth and membrane stabilization. We have shown that RBC stroma and lysate polyamines are significantly elevated in sickle cell disease (SS and SC) relative to controls (AA). The crude spectrin fraction of the red cell stroma has associated with it a large fraction of the total stroma polyamines, suggesting that the polyamines in the stroma are preferentially bound to spectrin. The high levels of polyamines associated with the spectrin of young and ISC's relative to normal may contribute to an alteration of the physical-chemical properties of the sickle RBC membrane. Spermine levels were high in the SS lysates but not detectable in SC or AA lysates. Levels in SC patients were intermediate. In preliminary experiments using washed red cells obtained from SS and AA subjects, there was extensive incorporation of 14C putrescine into red cell protein. Labelled N-(gamma-glutamyl) putrescine was isolated, consistent with covalent binding of the polyamines to protein.
D000755:Anemia, Sickle Cell / Q000097:blood*; D004913:Erythrocytes, Abnormal / Q000032:analysis*; D006441:Hemoglobin A / Q000032:analysis; D006444:Hemoglobin C / Q000032:analysis; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D008565:Membrane Proteins / Q000097:blood*; D011073:Polyamines / Q000097:blood*; D013049:Spectrin / Q000032:analysis; D013095:Spermidine / Q000097:blood; D013096:Spermine / Q000097:blood; D013789:Thalassemia / Q000097:blood
D006451:Hemoglobin, Sickle; D008565:Membrane Proteins; D011073:Polyamines; D013049:Spectrin; D013096:Spermine; D006444:Hemoglobin C; D006441:Hemoglobin A; D013095:Spermidine