Linking SOX3, SRY, and disorders of neurodevelopment

Abstract

SRY and SOX3 proteins are transcription factors from the SOX family. All the SOX genes contain a structurally homologous HMG domain sequence that binds and regulates DNA. In different literature examples, it has been shown that this HMG box is functionally interchangeable between SRY and other SOX proteins. In this chapter, we review actions and interactions of SOX3 and SRY in brain tissue and possible contributions to neurodevelopment. We suggest that, at early stages of neurodevelopment, the action of both transcription factors on the same gene targets and/or coactivators could interfere in cell fate decisions, rendering alterations in brain development. Thereby, boys will be more vulnerable to toxic stress exposition and/or other genetic factors acting on the epigenetic regulation of SRY and/or SOX3 proteins as well as acting on their gene targets.