An additional safety issue that remains to be investigated for the management of GM1 gangliosidosis patients undergoing β-gal augmentation therapy is the potential for continual β-gal delivery to lysosomes to disrupt a multienzyme complex that exists between protective protein cathepsin A (PPCA), neuraminidase 1 (Neu1), and β-gal (29). Here, NEU1 is linked to GM1 gangliosidosis.