TMEM237 is a transmembrane protein that functions as a critical component of the ciliary transition zone (TZ), the gatekeeper structure at the base of primary cilia 1. It is essential for proper ciliogenesis and localizes to the TZ where it interacts with other ciliopathy-associated proteins including RPGRIP1L/MKS5, MKS-2/TMEM216, B9D1, and B9D2 1. TMEM237 regulates Wnt signaling pathway function and participates in the MKS module that controls ciliary composition and TZ integrity 12. Loss of TMEM237 function results in defective ciliogenesis and Wnt pathway deregulation, as demonstrated in zebrafish gastrulation models 1. Mutations in TMEM237 cause Joubert syndrome 14 (JBTS14), a primary ciliopathy characterized by cerebellar and brainstem malformation 13. TMEM237 mutations are frequently associated with renal involvement in Joubert syndrome patients, requiring close monitoring of renal function 3. Additionally, TMEM237 may contribute to dopaminergic neurodegeneration through Wnt pathway dysfunction in response to environmental pesticide exposure 4. The mutation spectrum includes nonsense mutations, splicing variants, and copy number deletions 56.