Three mouse models of human thalassemia.
- 1 August 1981
- journal article
- research article
- Published by Proceedings of the National Academy of Sciences in Proceedings of the National Academy of Sciences
- Vol. 78 (8) , 5056-5060
- https://doi.org/10.1073/pnas.78.8.5056
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.This publication has 32 references indexed in Scilit:
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