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Publication : Knockout-transgenic mouse model of sickle cell disease.

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.
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