Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease.
To create mice expressing exclusively human sickle hemoglobin (HbS), transgenic mice expressing human alpha-, gamma-, and betaS-globin were generated and bred with knockout mice that had deletions of the murine alpha- and beta-globin genes. These sickle cell mice have the major features (irreversibly sickled red cells, anemia, multiorgan pathology) found in humans with sickle cell disease and, as such, represent a useful in vivo system to accelerate the development of improved therapies for this common genetic disease.
Pubmed ID: 9346488 RIS Download
Anemia, Sickle Cell | Animals | Disease Models, Animal | Female | Globins | Hemoglobin, Sickle | Humans | Male | Mice | Mice, Inbred C57BL | Mice, Knockout | Mice, Transgenic