This knock-in mouse model of sickle cell disease was generated by replacing the mouse β-globin genes with a DNA fragment containing the human Aγ-globin gene and a human βS-globin gene, and replacing the mouse α-globin genes with a human α-globin gene. At birth, these mice continue to synthesize significant amounts of human fetal hemoglobin (HbF) but switch to more than 99% sickle cell hemoglobin (HbS) during the first week of life and develop severe disease. Their HbF to HbS switch mimics that observed in human sickle cell patients. Blood smears from βS/βS mice show many rigid, elongated, sickle-shaped red blood cells, and erythroid progenitors are visible in the sinusoids, indicative of severe anemia. Mice have been described in:

Wu LC, Sun CW, Ryan TM, Pawlik KM, Ren J, and Townes TM. Correction of sickle cell disease by homologous recombination in embryonic stem cells. Blood, 2006; 108(4):1183-8

Mice are available from Jackson Laboratory: stock number 013071.


  • B6;129-Hbatm1(HBA)Tow
  • Hbbtm2(HBG1,HBB*)Tow/Hbbtm3(HBG1,HBB)Tow/J

UAB Reference: U2007-0037

For more information or to request a license, please contact:

Eve Vick, PhD
Licensing Manager
Phone: (205) 996-4740

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