SOX10 is a member of the SRY-related HMG-box family of transcription factors. It is required for the terminal differentiation of oligodendrocytes and myelination. In the peripheral nervous system, SOX10 maintains pluripotency of neural crest stem cells and suppresses neuronal differentiation.
The encoded protein may act as a transcriptional activator after forming a protein complex with other proteins. This protein acts as a nucleocytoplasmic shuttle protein and is important for neural crest and peripheral nervous system development. Mutations in this gene are associated with Waardenburg-Shah and Waardenburg-Hirschsprung disease.