SOX10 is a SRY-box transcription factor essential for neural crest-derived cell differentiation and myelination. Functionally, SOX10 acts as a DNA-binding transcription factor that binds DNA via its HMG domain and undergoes conformational changes to activate downstream transcription factors 1. In the central nervous system, SOX10 plays a central role in oligodendrocyte maturation and CNS myelination by activating myelin genes including MYRF and MBP [UniProt]. In the peripheral nervous system, SOX10 regulates Schwann cell differentiation and myelination programs 2. SOX10 also governs development of neural crest derivatives including melanocytes and enteric nervous system neurons 3. SOX10 mutations cause multiple developmental disorders. Heterozygous mutations cause Waardenburg syndrome types 2 and 4, and peripheral demyelinating neuropathy with central dysmyelination (PCWH), reflecting its role in melanocyte, glial, and enteric neuron development 3. In cancer, SOX10 regulates melanoma tumor growth and cell-state plasticity; low SOX10 expression promotes aggressive neural stem cell-like phenotypes linked to therapy resistance 4. SOX10 protein stability is regulated by p300 acetyltransferase activity, offering potential therapeutic targets 5. In schwannomas, novel SOX10 indel mutations impair myelination gene transactivation despite retained DNA binding, suggesting a differentiation-blocking mechanism 2.