Establishment of an adult model of β⁰-thalassemia.

(A) Cellulose acetate analysis showing the hemoglobin patterns in ED 14.5 Hbb^+/+, Hbb^th3/+, and Hbb^th3/th3 embryos. E1, E2, and E3 indicate the 3 embryonic hemoglobins. (B) Mice engrafted with Hbb^th3/th3 hematopoietic FLCs die 6 to 9 weeks after receiving transplants. Donor FLCs harvested from ED 14.5 embryos were injected in irradiated C57BL/6 recipient mice. Mice engrafted with Hbb^th3/th3 FLCs died or had to be killed 7 to 9 weeks after receiving transplants. Recipients of Hbb^+/+ and Hbb^th3/+ FLCs survived for at least 8 months (duration of follow-up). (C) Spleen sizes in mice engrafted with Hbb^+/+, Hbb^th3/+, and Hbb^th3/th3 FLCs at 6 to 8 weeks after receiving transplants.