CELSR3

CELSR3, or Flamingo-3, is the neuron-enriched member of the CELSR family of seven-pass atypical cadherins. Like CELSR1 and CELSR2 it carries a large ectodomain of cadherin repeats and EGF-like modules ahead of its GPCR-like membrane core, but its function is concentrated in the central nervous system. Genetic studies in mice show that Celsr3 is required for axon guidance in the forebrain and hindbrain, for maintenance of neural progenitor polarity, and for proper neuronal migration, acting through Vangl-Frizzled planar cell polarity machinery. Human CELSR3 variants have been reported in neurodevelopmental phenotypes, though disease causality remains under investigation.