POMT2 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan: Because Emx1Cre (and NestinCre) conditional deletion of Dag1 or Pomt2 leads to widespread loss of functional Dystroglycan in the forebrain in contrast with the previously studied NeuroD6Cre conditional deletion, which targets pyramidal neurons, these models more accurately model dystroglycanopathy.