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. Competes with PAX4 in binding to a common element in the glucagon, insulin and somatostatin promoters (By similarity). Regulates specification of the ventral neuron subtypes by establishing the correct progenitor domains. Acts as a transcriptional repressor of NFATC1-mediated gene expression (By similarity). Interacts with MAF and MAFB (By similarity). 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 (By similarity). Interacts with TLE6/GRG6 (By similarity). Expressed in the embryonic headfolds and to a lesser extent in the trunk of neurula at 10 dpc. Expressed in the embryonic headfolds and to a lesser extent in the trunk of neurula at 10 dpc. Up-regulated in response to transient cerebral ischemia in cerebral cortex, striatum and subcortical white matter fibers in the ischemic region starting from 24 hours after initiating the ischemia. Ubiquitinated by TRIM11, leading to ubiquitination and proteasomal degradation. 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.