CELSR3 (cadherin EGF LAG seven-pass G-type receptor 3) is an adhesion G protein-coupled receptor 1 with critical roles in nervous system development and cell signaling. As a planar cell polarity protein 2, CELSR3 functions in neuron migration, axon guidance, and neural circuit formation, working in concert with signaling partners like FZD3 and FZD6 3. The receptor guides axon development across central, peripheral, sympathetic, and enteric nervous systems 4, with particular importance in globus pallidus development and basal ganglia connectivity 5. CELSR3 also serves as a receptor for the secreted tumor suppressor Reprimo, activating the Hippo-YAP/TAZ-p73 apoptosis pathway 6. Biallelic CELSR3 variants cause central nervous system and urinary tract anomalies, with computational modeling suggesting protein structural variants affect disease penetrance 2. Recent studies identify CELSR3 as a risk factor for Tourette syndrome; Celsr3 mutant mice display tic-like behaviors mediated through altered striatal dopamine D3 receptor distribution 7. These findings establish CELSR3 as essential for developmental patterning and neurotransmitter signaling, with emerging therapeutic relevance for neuropsychiatric and neuroendocrine disorders 8.