Three mouse models of human thalassemia.

AUTOR(ES)

Martinell, J

RESUMO

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 three stocks of mice, each one heterozygous for one 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 approximately 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 one alpha-globin gene is deleted. Thalassemic mouse erythroid cells appear to compensate partially for the loss of half of their alpha-globin genes.

Documentos Relacionados

• Protein synthesis in a cell free human reticulocyte system: ribosome function in thalassemia.
• Removal of erythrocyte membrane iron in vivo ameliorates the pathobiology of murine thalassemia.
• Differing erythrocyte membrane skeletal protein defects in alpha and beta thalassemia.
• Sickle Cell Anemia and Thalassemia. A Primer for Health Care Professionals
• Demonstration of non-functional beta-globin mRNA in homozygous beta (0) thalassemia.