We recently discovered a relationship between ER dysfunction and neurological disorders by generating mice with central nervous system (CNS)-specific deletion of Derlin-1 (Derl1) or Derlin-2 (Derl2), which encode vital components of the ERAD and ER stress-induced preemptive quality control (ERpQC) systems12, and observed impaired neurite outgrowth due to disrupted cholesterol synthesis in neurons and progressive brain atrophy, primarily in the cerebellum13. Here, DERL2 is linked to Brain atrophy.