As for Pompe disease, targeted intralingual administration of an AAV9 encoding IGF2-tagged hGAA resulted in efficient glycogen clearance and reversal of pathology in lingual myofibers and hypoglossal (XII) motoneurons in a KO model (70); lingual weakness and hypoglossal neuropathology are common characteristics of Pompe disease (77–79). Here, IGF2 is linked to glycogen storage disease II.