Rax and Six-3 (also a transcription factor) maintain the forebrain's ability to secrete Shh by inhibiting activity of the signaling molecule Wnt.

[9] Mutations in this gene have been reported in patients with defects in ocular development, including microphthalmia, anophthalmia, and coloboma.

[10] Individuals who have a mutation in the RAX gene fail to develop ocular structures, referred to as anophthalmia.

In the frog Xenopus tropicalis, Rax mutants are eyeless; the future retinal tissue instead has diencephalon and telencephalon features.

[12][13] This article incorporates text from the United States National Library of Medicine, which is in the public domain.