Figure 1.
Figure 1. Hemoglobin switching and survival curve analyses of humanized CA mice. (A) Human fetal-to-adult globin gene switching was analyzed in humanized γHPFHβ0/γβA compound heterozygous mice after birth. Human γ-globin and β-globin chain levels in peripheral blood hemolysates were measured by HPLC and plotted as a percentage of total β-like chains across time. High fetal globin chain levels at birth are gradually replaced by adult chains during the first 5 weeks of life. (B) The fractional survival of fully humanized CA (α2α1/α2α1, γHPFHβ0/γHPFHβ0) and littermate control mice are plotted across time. The majority of CA mice die prior to weaning age with a median lifespan of 17 days, whereas all the control littermates survive into adulthood.

Hemoglobin switching and survival curve analyses of humanized CA mice. (A) Human fetal-to-adult globin gene switching was analyzed in humanized γHPFHβ0/γβA compound heterozygous mice after birth. Human γ-globin and β-globin chain levels in peripheral blood hemolysates were measured by HPLC and plotted as a percentage of total β-like chains across time. High fetal globin chain levels at birth are gradually replaced by adult chains during the first 5 weeks of life. (B) The fractional survival of fully humanized CA (α2α12α1, γHPFHβ0HPFHβ0) and littermate control mice are plotted across time. The majority of CA mice die prior to weaning age with a median lifespan of 17 days, whereas all the control littermates survive into adulthood.

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