TMEM97 is the recently identified σ2 receptor (Alon et al., 2017) that binds to the intracellular cholesterol trafficking protein NPC1 (Niemann-Pick disease, type C1), reducing its abundance and in turn cholesterol transfer to the ER (Ebrahimi-Fakhari et al., 2016). Here, NPC1 is linked to Niemann-Pick disease, type C1.