Similar to the age-dependent accumulation of dolichols in brains of the elderly,78,79 which was greatly increased in patients with neuronal ceroid lipofuscinosis,80,81 ATP5G1/2/3 accumulates in lipofuscin-like lipopigments inside normal neurons during aging, a process amplified in neuronal ceroid lipofuscinosis and other lysosomal disorders.82 Interestingly, these previously reported observations are consistent with the LC-MS detection of this protein specifically in TIS-NM samples, which can be associated to the early stages of NM-containing organelle formation. The gene discussed is ATP5MC1; the disease is lysosomal storage disease.