Comparative role of SOX10 gene in the gliogenesis of central, peripheral, and enteric nervous systems

• Published in: Differentiation (London) Vol. 128; pp. 13 - 25
• Main Authors: Bhattarai, Chacchu, Poudel, Phanindra P., Ghosh, Arnab, Kalthur, Sneha G.
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 01-11-2022
• Subjects: Enteric Nervous System - metabolism; Gliogenesis; High Mobility Group Proteins - genetics; High Mobility Group Proteins - metabolism; Hirschsprung Disease - genetics; Humans; Neural Crest; Neural crest cells; Neuroglia; PCWH; SOX10 gene; SOXE Transcription Factors - genetics; SOX10 gene; PCWH; Gliogenesis; Neuroglia; Neural crest cells
• ISSN: 0301-4681; 1432-0436
• DOI: 10.1016/j.diff.2022.09.001

SOX10 gene and SOX10 protein are responsible for the gliogenesis of neuroglia from the neural crest cells. Expression of SOX10 gene encodes SOX10 protein which binds with DNA at its minor groove via its HMG domain upon activation. SOX10 protein undergoes bending and changes its conformation after binding with DNA. Via its transactivation domain and HMG domain, it further activates several other transcription factors, these cause gliogenesis of the neural crest cells into neuroglia. In literature, it is stated that the SOX10 gene helps in the formation of schwann cells, oligodendrocytes, and enteric ganglia from neural crest cells. Altered expression of the SOX10 gene results in agliogenesis, dysmyelination, and demyelination in the nervous system as well as intestinal aganglionosis. This review highlighted that there is a role of the SOX10 gene and SOX10 protein in enteric gliogenesis from the neural crest cells.
•SOX10 gene is essential for the differentiation of neural crest cells into neuroglia.•SOX10 gene plays a role in the development of enteric neuroglia.•Its mutation results in demyelination, Waardenburg syndrome, and Hirschsprung's disease.