VANGL2 is involved in the control of early morphogenesis and patterning of both axial midline structures and the development of neural plate. It plays a role in the regulation of planar cell polarity, particularly in the orientation of stereociliary bundles in the cochlea. It is required for polarization and movement of myocardializing cells in the outflow tract and seems to act via RHOA signaling to regulate this process. The VANGL2 gene encodes a homolog of Drosophila 'strabismus/Van Gogh' (Stbm/Vang), a component of the frizzled-dishevelled tissue polarity pathway.[supplied by OMIM]
