Chimeric Dag1-null mice exhibit reduced levels of AChE at the NMJ (Côté et al., 1999; Jacobson et al., 2001); however, a functional consequence of AChE disruption has not yet been shown in the α-dystroglycanopathies or its models. This evidence concerns the gene DAG1 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.