Steroidogenic organ development and homeostasis: A WT1-centric view

• The Wilms' tumor suppressor WT1 is a key regulator of adrenogonadal development.
• Alternatively spliced isoforms fulfill distinct molecular and cellular functions.
• Adrenogonadal progenitors persist in adult life.
• Progenitors share similar differentiation pathways in development and tissue homeostasis.

Adrenal and gonads are the main steroidogenic organs and are central to regulate body homeostasis in the vertebrate organism. Although adrenals and gonads are physically separated in the adult organism, both organs share a common developmental origin, the adrenogonadal primordium. One of the key genes involved in the development of both organs is the Wilms' tumor suppressor WT1, which encodes a zinc finger protein that has fascinated the scientific community for more than two decades. This review will provide an overview of the processes leading to the development of these unique organs with a particular focus on the multiple functions WT1 serves during adrenogonadal development. In addition, we will highlight some recent findings and open questions on how maintenance of steroidogenic organs is achieved in the adult organism.