CLCN7 and lysosomal storage disease: We note that ATP5G1/2/3, that we found in NM-containing organelles, is the primary marker of broad range of neuronal ceroid lipofuscinoses (i.e., CLN2, -3, -4, -5, -6, -7, -8, -9, CLCN7).36 ATP5G1/2/3 accumulates in autophagic vacuoles and lysosomes of neurons where autophagy or some lysosomal enzymes are blocked, as in lysosomal storage disorders.76,77 Intriguingly, this protein also accumulates inside autophagic vacuoles in normal aged mice,76 consistent with a decline of autophagic-lysosomal function during normal aging.