DAG1 and muscle-eye-brain disease: Furthermore, taken with the data in Figure 1B–D, they illustrate that the severity of the cortical migration phenotype scales with the degree of Dystroglycan hypoglycosylation; Emx1Cre:Dag1 cKOs and Emx1Cre:Pomt2 cKOs model a severe form of dystroglycanopathy (Walker-Warburg Syndrome, Muscle-Eye-Brain disease) and B4gat1M155T/M155T and FkrpP448L/P448L mutants modeling a milder form of the disease.