Mutations in ATP13A2, GBA1 and VPS35 have been associated with PD.9, 10, 11, 12 Mutations in progranulin and charged multivesicular body protein 2B (CHMP2B) have been identified as genetic causes of amyotrophic lateral sclerosis and frontotemporal dementia.13, 14, 15 Postmortem brain tissues of neurodegenerative diseases have exhibited deposition of endosomal and autophagic vesicles.16 Therefore, neurodegenerative proteinopathies might be attributed to lysosomal dysfunction. This evidence concerns the gene CHMP2B and amyotrophic lateral sclerosis.