title: Radiation-induced alpha-thalassemia in mice.
abstract: The clinical hematologic change in 2 groups of progeny from mice carrying radiation-induced strain SEC alpha-chain deficiencies was found to be similar to the hematologic alterations in persons with alpha-thalassemia. The heterozygous deletion or inactivation of the alpha-chain gene in mice caused an anemia similar to alpha-thalassemina minor in persons. The alpha-chain deficiency in mice created an erythrocytosis, reticulocytosis, and microcytic, hypochromic anemia comparable with the changes in human alpha-thalassemia minor resulting from deletion of the alpha-chain gene. These mouse mutants are the only known animal models of human thalassemia. A comparison of hematologic values obtained from progeny possessing an alpha-chain gene deficiency and from progeny possessing a beta-chain duplication suggested that the deficiency of alpha-chain synthesis, rather than a simple imbalance between the amounts of alpha- and beta-chains produced, was primarily responsible for the altered hematologic characteristics in these alpha-thalassemic mice.
mesh_terms: D000818:Animals; D001772:Blood Cell Count; D004195:Disease Models, Animal; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D008297:Male; D051379:Mice / Q000097:blood*; D009154:Mutation; D011833:Radiation Injuries, Experimental / Q000150:complications; D012156:Reticulocytes; D012995:Solubility; D013789:Thalassemia / Q000097:blood* / Q000209:etiology
chemical_list: D006454:Hemoglobins