Since JNCL is a lysosomal storage disorder with alterations in endosomal/lysosomal system, the localization of the intracellular CTX in lysosomes was studied using staining with anti-LAMP-1 antibodies in CTX-A488 labeled WT and Cln3Δex7/8 cells (Figure 5). The gene discussed is LAMP1; the disease is lysosomal storage disease.