Efficient engraftment of pluripotent stem cell-derived myogenic progenitors in a novel immunodeficient mouse model of limb girdle muscular dystrophy 2I

摘要

Characterization of FKRP -NSG mice. Representative plots show FACS profile for B (CD19), T (CD3), and NK (CD49b/NK1.1) cells in the peripheral blood of B6 (control), FKRP , and FKRP -NSG mice. Western blot for IIH6 in TA muscle lysates from 10-week-old B6, FKRP , NSG, and FKRP -NSG mice. β-DG was used as loading control. Western blot for IIH6 in TA, triceps (Tri), gastrocnemius (Gas), quadriceps (Quad), diaphragm (Dia), and heart lysates from 7-week-old B6 and FKRP -NSG mice. β-DG was used as loading control. Representative images show IIH6 immunostaining in TA, Tri, Gas, Quad, and Dia muscles from 7-week-old B6 and FKRP -NSG mice. DAPI stained nuclei. Scale bar is 100 μm