VSX2 is a transcriptional regulator that binds DNA at the consensus sequence 5'-[TC]TAATT[AG][AG]-3' 1 and serves as a master regulator of retinal development. In the retina, VSX2 marks multipotent retinal progenitor cells (RPCs) throughout development 2 and is essential for eye organogenesis, with mutations causing congenital microphthalmia 3. VSX2 functions through its homeodomain and CVC domain to maintain high-affinity DNA binding required for retinal progenitor identity and proliferation 3. During retinal differentiation, VSX2-positive cells progress from RPCs to bipolar cells and Müller glia 4. The gene operates through stage- and cell-type-specific super-enhancer modules that regulate its complex expression pattern 5. Dysregulation of VSX2 enhancer modules causes distinct retinal defects: one module deletion leads to microphthalmia through reduced RPC proliferation, while another deletion specifically disrupts bipolar neuron development without affecting overall retinal architecture 6. Beyond retina, VSX2 marks V2a interneurons in spinal cord, where these cells demonstrate therapeutic potential for spinal cord injury repair 7. VSX2 mutations in humans cause microphthalmia and coloboma diseases, establishing direct links between transcriptional dysfunction and congenital ocular malformations.