The zebrafish has become a valuable disease model for dystroglycanopathy as mouse KOs for dystroglycan (Dag1) and for the glycosyltransferases initiating O-mannosylation, Pomt1 and Pomt2, show early embryonic lethality due a rodent-specific disruption in Reichert’s membrane before placental formation [25–27]. Here, POMT1 is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.