Changes to the DGC, such as due to aberrant glycosylation of α-dystroglycan (associated with Fukuyama-type congenital muscular dystrophy), or absence of components of the DGC, such as α-dystrobrevin, dystroglycan (associated with dystroglycanopathy, limb girdle muscular dystrophy and congenital muscular dystrophy) [30] or syntrophin, results in altered NMJ morphology and function [21,31,48,49,50,51]. The gene discussed is DAG1; the disease is congenital muscular dystrophy due to LMNA mutation.