SRY (Sex Determining Region Y)-Box 10 or SOX-10, is a nuclear transcription factor that acts in regulation of embryonic development and in the specification and differentiation of cells of melanocytic lineage. It is a valuable tool to detect for SOX-10 expression in tissues in which SOX-10 has been reported to diffusely be expressed in carcinomas such as neurofibroma and schwannoma. While mutations in the SOX-10 gene have been linked to diseases: Waardenburg-Shah disease and Waardenburg-Hirschsprung disease.