VAX2 is a 290 amino acid homeobox protein that plays a crucial role in development of the eye, particularly in the specification of the ventral optic vesicle and in establishment of a correct dorsoventral pattern. VAX2 acts as a transcription factor with VAX1 to cooperatively regulate retinal differentiation, neuroepithelial cell proliferation and axial polarization in the retina. Together, VAX1 and VAX2 repress transcription of Pax-6, a strong inducer of retinal development. VAX1 and VAX2 localize to the nucleus and contain one homeobox DNA-binding domain each.