Transcription factor with important functions in the development of the eye, nose, central nervous system and pancreas. Required for the differentiation of pancreatic islet alpha cells (PubMed:9163426). Competes with PAX4 in binding to a common element in the glucagon, insulin and somatostatin promoters. Regulates specification of the ventral neuron subtypes by establishing the correct progenitor domains (By similarity). Acts as a transcriptional repressor of NFATC1-mediated gene expression (PubMed:23990468). Interacts with MAF and MAFB (PubMed:17901057). Interacts with TRIM11; this interaction leads to ubiquitination and proteasomal degradation, as well as inhibition of transactivation, possibly in part by preventing PAX6 binding to consensus DNA sequences (PubMed:18628401). Interacts with TLE6/GRG6 (PubMed:23990468). Expressed in osteoclasts. Dominant isoform expressed in the eye, including in the retina and cornea (PubMed:21084637). Abundantly expressed in the lens epithelium (PubMed:21084637). Dominant isoform expressed in the eye, including in the retina and cornea (PubMed:21084637). Weakly expressed in the lens epithelium (PubMed:21084637). Expressed in the developing eye, nose, brain and pancreas (PubMed:21084637). At 9 dpc, expressed in the telencephalon, diencephalon, neural tube, optic vesicle and pancreas. Throughout development, expression continues in the dorsal and ventral pancreas. Expressed during cortical neurogenesis from 11 to 18 dpc. High levels in the early radial glial progenitors from 11 to 14 dpc and gradually decrease thereafter (at protein level). During corticogenesis, the protein level declines faster than that of the mRNA, due to proteasomal degradation (PubMed:18628401). Abundantly expressed in the newborn eye. Expressed in the developing eye at 9.5 dpc, the expression becomes much stronger and additionally expressed in the neural tube and optic and lens vesicles at 11.5 dpc. Expression is then reduced by 12.5 dpc and becomes significantly decreased from 14.5 dpc to 18.5 dpc of embryonic development. Significantly expressed in the newborn eye. Induced by TNFSF11/RANKL-induced osteoclastogenesis. Ubiquitinated by TRIM11, leading to ubiquitination and proteasomal degradation. Sumoylated by SUMO1 at 'Lys-91'. Defects in Pax6 are the cause of a condition known as small eye (Sey) which results in the complete lack of eyes and nasal primordia. Belongs to the paired homeobox family. Truncated N-terminus.