MCOLN1 and central nervous system cancer: In this regard, since the primary source of endogenous ROS are mitochondria, treating glioma cells with carbonyl cyanide m-chlorophenylhydrazone (CCCP), that is able to induce oxidative stress (33), stimulates a TRPML1-dependent autophagy, as demonstrated by the ability of the autophagic inhibitor bafilomycin-A1, the TRPML1 inhibitor sphingomyelin, and MCOLN1 silencing, to inhibit the CCCP-mediated effects (11).