Wilms' Tumor 1 (WT1) is a transcription factor named from Wilms' Tumor 1, an embryonal malignancy of the kidneys that is caused by mutations in the WT1 gene. It is highly important in development, particularly of the genitourinary system, and mutations and dysregulation of expression of WT1 result in a variety of syndromes affecting the genitourinal system and other tissues. WT1 has myriad biological functions and a host of interacting partners and target genes. It can behave as a transcriptional activator, or a repressor, and can act as an oncogene or a tumor suppressor.