The lack of observed ciliopathy or Shh-related phenotypes outside the brain (e.g., renal cysts, skeletal dysplasia, or limb and digit abnormalities) in our KO mice suggests specific importance of TMEM161B expression in the developing CNS, or that the developing CNS could be specifically vulnerable to a loss of TMEM161B in a way other tissues or developmental stages are not. Here, TMEM161B is linked to ciliopathy.