Figure 12.

Quantification of muscle morphometry and lectin precipitation in GALGT2-overexpressing WT, mdx, and dystroglycan-deficient skeletal muscles. A and B: Percentages of myofibers with centrally located nuclei (A) and variance in myofiber diameter (B) were measured for rAAV1.CMV.Galgt2-treated and mock-treated muscles from P3Pro-Cre Dag1^lox/lox mice. Line at 250 (or below) indicates variance levels expected for nondystrophic muscle, whereas normal percentages of central nuclei are typically below 5%. In this mouse strain, gastroc, TA, and quad muscles fail to express dystroglycan, whereas triceps muscles express dystroglycan. C: Protein lysate (150 μg) extracted in 1% NP-40 was precipitated with WFA or WGA. Precipitated proteins were immunoblotted for α dystroglycan (using IIH6), β dystroglycan (using 43DAG), or the CT glycan (using CT2). The gastroc muscles from WT, mdx, or P3ProCreDag1^lox/lox (Dag1^−/−) mice that had been treated with rAAV1.CMV.Galgt2 for 0, 3, or 12 weeks are compared. Error bars represent SEM (A and B). n = 4 to 8 muscles per condition (A and B). CMV, cytomegalovirus; CT, cytotoxic T cell; GALGT2, β-1,4 N-acetylgalactosaminyltransferase 2; gastroc, gastrocnemius; IIHA, IIH6, natively glycosylated α dystroglycan; quad, quadriceps; rAAV, recombinant adeno-associated virus; TA, tibialis anterior; WFA, Wisteria floribunda agglutinin; WGA, wheat germ agglutinin; WT, wild-type.