DERL1 and nervous system disorder: 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.