Because perlecan-null mice show complete absence of AChE at the NMJ, it is possible that neuromuscular transmission in α-dystroglycanopathies may also be impaired by a disruption of an AChE-perlecan-αDG complex (Arikawa-Hirasawa et al., 2002). Here, HSPG2 is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.