Rapid Publication Effect of Human #5-Globin Chains on Cellular Properties of Red Cells from #l-Thalassemic Mice

The transgenic mouse system provides an in vivo setting in which to examine the effects on mouse red cells of hemoglobin genes that have been genetically introduced into the animals' genome. In this report, we have analyzed the cellular proper-ties of red cells from homozygous f3-thalassemic mice (Hbb"'/Hbb"'), homozygous,B-thalassemic transgenic mice containing a human 13-sickle (18) gene (Hbb"l/Hbbth-l + 08), and normal animals. The presence of human #'-globin chains in red cells from the Hbb"'/Hbbt" ' +,13 transgenic animals was noted to have a significant effect on cellular deformability and density distribution, as well as on the degree of anemia in these animals. We conclude from these studies that red cell deformability and density distribution is a sensitive means for assessing at the cellular level the effects of globin genes geneti-cally introduced into whole organisms. In addition, these stud-ies suggest that small decreases in the amount of excess a-glo-bin chains can significantly ameliorate the severity of anemia in the fl-thalassemic mouse