All GM2 gangliosidosis disease models showed significant accumulation of endolysosomal proteins including degradative enzymes (such as LIPA and CTSD), membrane pumps (SLC37A3 and LMBRD1), lipid transporters (NPC1/2, SCARB2 and ABCA3), integral limiting membrane proteins (LAMP1), modulators of lysosomal position (RNF167 and TMEM106B) and exosomal markers (TSPAN3/6/7 and CD63) (Fig 2C and S2 Table). Here, LIPA is linked to GM2 gangliosidosis.