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Pax-6 (Paired box protein) is a transcription factor with important functions in the development of the eye, nose, central nervous system and pancreas. Pax-6 is 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. Pax-6 regulates specification of the ventral neuron subtypes by establishing the correct progenitor domains. Defects in Pax-6 are associated with a number of diseases and abnormalities of the eye and optic nerve.