We showed that terminal ANCL patients exhibit lysosomal dysfunction, including increased levels of LAMP1, LAMP2 and V-ATPase B1/2 protein, along with significant secondary elevations in the activity of the lysosomal enzymes PPT1, β-glucuronidase (β-gluc), and β-hexosaminidase (β-Hexa) in multiple brain regions89. Here, LAMP1 is linked to adult neuronal ceroid lipofuscinosis.