First Author | Ryan TM | Year | 1997 |
Journal | Science | Volume | 278 |
Issue | 5339 | Pages | 873-6 |
PubMed ID | 9346487 | Mgi Jnum | J:44160 |
Mgi Id | MGI:1099412 | Doi | 10.1126/science.278.5339.873 |
Citation | Ryan TM, et al. (1997) Knockout-transgenic mouse model of sickle cell disease [see comments]. Science 278(5339):873-6 |
abstractText | When transgenic mice that expressed human sickle hemoglobin were mated with mice having knockout mutations of the mouse alpha-and beta-globin genes, animals were produced that synthesized only human hemoglobin in adult red blood cells. Similar to many human patients with sickle cell disease, the mice developed a severe hemolytic anemia and extensive organ pathology. Numerous sickled erythrocytes were observed in peripheral blood. Although chronically anemic, most animals survived for 2 to 9 months and were fertile. Drug and genetic therapies can now be tested in this mouse model of sickle cell disease. |