Figure 6.

Intramuscular injection of 2 × 10¹¹ vg of AAV1-COLQ-IRES-EGFP into the left anterior tibial muscle of Colq^−/− mice. (a) Acetylcholinesterase (AChE) activity and collagen Q (ColQ) are colocalized to the acetylcholine receptors (AChR) in the injected muscle, as well as in the noninjected triceps muscle, although the signal intensities are not as high as those of the injected muscle. In contrast to ColQ, an intracellular molecule, enhanced green fluorescent protein (EGFP), is expressed only in the injected muscle, but not in the noninjected muscle. Bar = 10 µm. (b) Quantification of globular and ColQ-tailed AChE species of skeletal muscles (mean and SD, n = 4). In the injected left hindlimb, the activity of ColQ-tailed AChE is similar to that of wild type. In the noninjected both forelimbs and right hindlimb, the activities are 21.5 and 28.4% of wild type, respectively. AAV1, adeno-associated virus serotype 1.