SOX10 (SRY-box transcription factor 10) is a critical transcription factor expressed in neural crest-derived cells and glial lineages throughout the nervous system[1]. SOX10 plays an essential role in the development and maintenance of peripheral glial cells, including Schwann cells, satellite glial cells, and enteric glial cells[2]. While SOX10 is primarily associated with glial cell development, it is also expressed in certain neuronal populations, particularly during development and in specific neural circuits. Mutations in the SOX10 gene are associated with several human diseases, including Hirschsprung disease, peripheral neuropathies, and disorders of the enteric nervous system.
SOX10 is a member of the SOX (SRY-related HMG-box) family of transcription factors, which play crucial roles in cell fate determination, differentiation, and maintenance[3]. During embryonic development, SOX10 is expressed in neural crest cells, which give rise to diverse cell types including peripheral neurons, glial cells, and melanocytes. In the adult nervous system, SOX10 continues to be expressed in glial cells where it maintains the identity and function of these essential supporting cells.
The study of SOX10-expressing cells has become increasingly important in neurodegenerative disease research, particularly in the context of Parkinson's disease, where the enteric nervous system and peripheral nervous system involvement are recognized as early features of the disease process[4].
SOX10-expressing cells are found in several locations throughout the nervous system:
SOX10-expressing cells serve multiple essential functions[5]:
SOX10-expressing cells are increasingly recognized in PD research[6]:
SOX10 operates through several key pathways[7]:
SOX10 represents a potential therapeutic target[8]:
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Britsch, S. et al. (2001). The transcription factor Sox10 is a key regulator of peripheral glial development. Genes & Development, 15(1), 66-78 ↩︎
Wegner, M. (2000). Transcriptional programming in the nervous system. Biochemical Society Transactions, 28(4), 343-350 ↩︎
Rao, M. et al. (2019). Enteric glia in Parkinson's disease. Nature Reviews Neurology, 15(12), 694-705 ↩︎
Sock, E. et al. (2001). Sox10 is required for the development of peripheral glia. Current Opinion in Neurobiology, 11(4), 443-449 ↩︎
Claire, M.B. et al. (2021). Enteric glia and Parkinson's disease. Journal of Parkinson's Disease, 11(2), 545-560 ↩︎
Jessen, K.R. & Mirsky, R. (2019). The origin and development of glial cells in peripheral nerves. Nature Reviews Neuroscience, 20(9), 547-562 ↩︎
Ferner, R.E. & Gutmann, D.H. (2013). Peripheral neuropathy and schwannoma: SOX10 and beyond. Brain, 136(5), 1372-1383 ↩︎