Human sickle hemoglobin in transgenic mice

Academic Article

Abstract

  • DNA molecules that contain the human α- and βs-globin genes inserted downstream of erythroid-specific, deoxyribonudease I super-hypersensitive sites were coinjected into fertilized mouse eggs and a transgenic mouse line was established that synthesizes human sickle hemoglobin (Hb S). These animals were bred to β-thalassemic mice to reduce endogenous mouse globin levels. When erythrocytes from these mice were deoxygenated, greater than 90 percent of the cells displayed the same characteristic sickled shapes as erythrocytes from humans with sickle cell disease. Compared to controls the mice have decreased hematocrits, elevated reticulocyte counts, lower hemoglobin concentrations, and splenomegaly, which are all indications of the anemia associated with human sickle cell disease.
  • Published In

  • Science  Journal
  • Digital Object Identifier (doi)

    Author List

  • Ryan TM; Townes TM; Reilly MP; Asakura T; Palmiter RD; Brinster RL; Behringer RR
  • Start Page

  • 566
  • End Page

  • 568
  • Volume

  • 247
  • Issue

  • 4942