We recently reported that the charged multivesicular body protein 2B (CHMP2B) mutation in the FTD/ALS–type 7–related gene inhibits neurite outgrowth; the CHMP2B mutant accumulates mutant protein in the Golgi and activates the Golgi stress signal Arf4. Here, CHMP2B is linked to amyotrophic lateral sclerosis.