Three mouse models of human thalassemia.

Abstract
Three types of mice with globin gene mutations, called 352HB, 27HB and Hbath-J, appear to be true animal models of human thalassemia. Expression of the .alpha.-globin genes in 3 stocks of mice, each one heterozygous for 1 of the .alpha.-globin mutations, was examined at the polypeptide, RNA and DNA levels. .alpha.-Globin polypeptide chains, relative to .beta.-globin chains in heterozygous thalassemic mice, are present at .apprx. 80% of normal. The ratios of .alpha.-globin to .beta.-globin RNA sequences are also 75-80% of normal, exactly reflecting the .alpha.-globin to .beta.-globin chain ratios. In the case of mutant 352HB, at least 1 .alpha.-globin gene is deleted. Thalassemic mouse erythroid cells appear to compensate partially for the loss of half of their .alpha.-globin genes.