Gene Symbol: Sox10
Description: SRY box 10
Alias: transcription factor SOX-10, SRY (sex determining region Y)-box 10, SRY-box containing gene 10
Species: rat
Products:     Sox10

Top Publications

  1. Roh J, Cho E, Seong I, Limb J, Lee S, Han S, et al. Down-regulation of Sox10 with specific small interfering RNA promotes transdifferentiation of Schwannoma cells into myofibroblasts. Differentiation. 2006;74:542-51 pubmed
    ..Importantly, we induce the transdifferentiation by down-regulating a single transcription factor, Sox10, thereby identifying a key molecular event in this process...
  2. Jagalur N, Ghazvini M, Mandemakers W, Driegen S, Maas A, Jones E, et al. Functional dissection of the Oct6 Schwann cell enhancer reveals an essential role for dimeric Sox10 binding. J Neurosci. 2011;31:8585-94 pubmed publisher
    ..For the first time in vivo, the dimeric Sox10 binding to this element is shown to be essential for enhancer activity, whereas monomeric Sox10 binding is ..
  3. Kellerer S, Schreiner S, Stolt C, Scholz S, Bösl M, Wegner M. Replacement of the Sox10 transcription factor by Sox8 reveals incomplete functional equivalence. Development. 2006;133:2875-86 pubmed
    Sox8 and Sox10 are two closely related transcription factors of the Sox protein family with overlapping expression patterns during development...
  4. Ito Y, Wiese S, Funk N, Chittka A, Rossoll W, Bommel H, et al. Sox10 regulates ciliary neurotrophic factor gene expression in Schwann cells. Proc Natl Acad Sci U S A. 2006;103:7871-6 pubmed
    ..domain class 3, transcription factor 1 (Oct6/SCIP/Tst-1) and paired box gene 3 (Pax3), SRY-box-containing gene 10 (Sox10) induces Cntf expression in Schwann cells...
  5. Kao S, Wu H, Xie J, Chang C, Ranish J, Graef I, et al. Calcineurin/NFAT signaling is required for neuregulin-regulated Schwann cell differentiation. Science. 2009;323:651-4 pubmed publisher
    ..Purification of NFAT protein complexes shows that Sox10 is an NFAT nuclear partner and synergizes with NFATc4 to activate Krox20, which regulates genes necessary for ..
  6. Stolt C, Rehberg S, Ader M, Lommes P, Riethmacher D, Schachner M, et al. Terminal differentiation of myelin-forming oligodendrocytes depends on the transcription factor Sox10. Genes Dev. 2002;16:165-70 pubmed
    b>Sox10 is a high-mobility-group transcriptional regulator in early neural crest. Without Sox10, no glia develop throughout the peripheral nervous system...
  7. Kuhlbrodt K, Herbarth B, Sock E, Hermans Borgmeyer I, Wegner M. Sox10, a novel transcriptional modulator in glial cells. J Neurosci. 1998;18:237-50 pubmed
    ..Here, we report on Sox10, a novel protein with predominant expression in glial cells of the nervous system...
  8. Stolt C, Lommes P, Friedrich R, Wegner M. Transcription factors Sox8 and Sox10 perform non-equivalent roles during oligodendrocyte development despite functional redundancy. Development. 2004;131:2349-58 pubmed
    ..Sox9 is involved in oligodendrocyte specification, whereas Sox10 is required for terminal differentiation...
  9. Schlierf B, Lang S, Kosian T, Werner T, Wegner M. The high-mobility group transcription factor Sox10 interacts with the N-myc-interacting protein Nmi. J Mol Biol. 2005;353:1033-42 pubmed
    The high-mobility group transcription factor Sox10 exerts many different roles during development of the neural crest and nervous system. To unravel its complex transcriptional functions, we have started to look for interaction partners...

More Information


  1. Hornig J, Fröb F, Vogl M, Hermans Borgmeyer I, Tamm E, Wegner M. The transcription factors Sox10 and Myrf define an essential regulatory network module in differentiating oligodendrocytes. PLoS Genet. 2013;9:e1003907 pubmed publisher
    ..In both cell types the transcription factor Sox10 is an essential component of the myelin-specific regulatory network...
  2. Zhang H, Chen H, Luo H, An J, Sun L, Mei L, et al. Functional analysis of Waardenburg syndrome-associated PAX3 and SOX10 mutations: report of a dominant-negative SOX10 mutation in Waardenburg syndrome type II. Hum Genet. 2012;131:491-503 pubmed publisher
    ..WS is classified into four subtypes (WS1-WS4) based on additional symptoms. PAX3 and SOX10 are two transcription factors that can activate the expression of microphthalmia-associated transcription factor (..
  3. Jiao Z, Zhang Z, Hornyak T, Hozeska A, Zhang R, Wang Y, et al. Dopachrome tautomerase (Dct) regulates neural progenitor cell proliferation. Dev Biol. 2006;296:396-408 pubmed
    ..Dct transcription is regulated directly or synergistically by Pax3, Sox10 and microphthalmia transcription factor (MITF)...
  4. Maka M, Stolt C, Wegner M. Identification of Sox8 as a modifier gene in a mouse model of Hirschsprung disease reveals underlying molecular defect. Dev Biol. 2005;277:155-69 pubmed
    Mice carrying heterozygous mutations in the Sox10 gene display aganglionosis of the colon and represent a model for human Hirschsprung disease...
  5. Liu Q, Melnikova I, Hu M, Gardner P. Cell type-specific activation of neuronal nicotinic acetylcholine receptor subunit genes by Sox10. J Neurosci. 1999;19:9747-55 pubmed
    The regulatory factor Sox10 is expressed in neural crest derivatives during development as well as in the adult CNS and peripheral nervous system...
  6. Pingault V, Bondurand N, Kuhlbrodt K, Goerich D, Prehu M, Puliti A, et al. SOX10 mutations in patients with Waardenburg-Hirschsprung disease. Nat Genet. 1998;18:171-3 pubmed
    ..The identification of Sox10 as the gene mutated in Dom mice (B.H. et al...
  7. Magnaghi V, Ballabio M, Roglio I, Melcangi R. Progesterone derivatives increase expression of Krox-20 and Sox-10 in rat Schwann cells. J Mol Neurosci. 2007;31:149-57 pubmed
  8. Uggenti C, De Stefano M, Costantino M, Loreti S, Pisano A, Avallone B, et al. M2 muscarinic receptor activation regulates Schwann cell differentiation and myelin organization. Dev Neurobiol. 2014;74:676-91 pubmed publisher
    ..g., Sox10 and Krox20) and downregulates proteins involved in the maintenance of the undifferentiated state (e.g...
  9. Owens S, Broman K, Wiltshire T, Elmore J, Bradley K, Smith J, et al. Genome-wide linkage identifies novel modifier loci of aganglionosis in the Sox10Dom model of Hirschsprung disease. Hum Mol Genet. 2005;14:1549-58 pubmed
    ..b>SOX10 mutations contribute to syndromic HSCR cases and Sox10 alleles in mice exhibit aganglionosis and pigmentary ..
  10. Sinner D, Kordich J, Spence J, Opoka R, Rankin S, Lin S, et al. Sox17 and Sox4 differentially regulate beta-catenin/T-cell factor activity and proliferation of colon carcinoma cells. Mol Cell Biol. 2007;27:7802-15 pubmed
    ..These findings indicate that Sox proteins can act as both antagonists and agonists of beta-catenin/TCF activity, and this mechanism may regulate Wnt signaling responses in many developmental and disease contexts. ..
  11. Hoff S, Zeller F, Von Weyhern C, Wegner M, Schemann M, Michel K, et al. Quantitative assessment of glial cells in the human and guinea pig enteric nervous system with an anti-Sox8/9/10 antibody. J Comp Neurol. 2008;509:356-71 pubmed publisher
    ..This is a comprehensive set of quantitative EGC measures in man and guinea pig that provides a basis for pathological assessment of glial proliferation and/or degeneration in the diseased gut...
  12. Agarwal P, Verzi M, Nguyen T, Hu J, Ehlers M, McCulley D, et al. The MADS box transcription factor MEF2C regulates melanocyte development and is a direct transcriptional target and partner of SOX10. Development. 2011;138:2555-65 pubmed publisher
    ..For example, mutations in SOX10 result in a severe form of Waardenburg syndrome, Type IV, also known as Waardenburg-Hirschsprung disease, ..
  13. Pingault V, Bodereau V, Baral V, Marcos S, Watanabe Y, Chaoui A, et al. Loss-of-function mutations in SOX10 cause Kallmann syndrome with deafness. Am J Hum Genet. 2013;92:707-24 pubmed publisher
    Transcription factor SOX10 plays a role in the maintenance of progenitor cell multipotency, lineage specification, and cell differentiation and is a major actor in the development of the neural crest...
  14. Fogarty E, Brewer M, Rodriguez Molina J, Law W, Ma K, Steinberg N, et al. SOX10 regulates an alternative promoter at the Charcot-Marie-Tooth disease locus MTMR2. Hum Mol Genet. 2016;25:3925-3936 pubmed publisher
    ..The transcription factor SOX10 is critical for Schwann cell development and maintenance, and many SOX10 target genes encode proteins required for ..
  15. Tomazelli K, Modolo F, Trentin A, Garcez R, Biz M. Temporo-spatial analysis of Osterix, HNK1 and Sox10 during odontogenesis and maxillaries osteogenesis. Tissue Cell. 2015;47:465-70 pubmed publisher
    ..Immunohistochemistry for MSC markers (Osterix) and NC cells (Sox10, HNK1) was performed...
  16. Fujiwara S, Hoshikawa S, Ueno T, Hirata M, Saito T, Ikeda T, et al. SOX10 transactivates S100B to suppress Schwann cell proliferation and to promote myelination. PLoS ONE. 2014;9:e115400 pubmed publisher
    ..We investigated the role of the transcription factor sex determining region Y (SRY)-box 10 (SOX10) in the proliferation and myelination of Schwann cells...
  17. Friedrich R, Schlierf B, Tamm E, Bösl M, Wegner M. The class III POU domain protein Brn-1 can fully replace the related Oct-6 during schwann cell development and myelination. Mol Cell Biol. 2005;25:1821-9 pubmed
    ..Similar to Oct-6, Brn-1 down-regulated its own expression at later stages of myelination. Thus, class III POU domain proteins can fully replace each other in Schwann cell development. ..
  18. Smit D, Smith A, Parsons P, Muscat G, Sturm R. Domains of Brn-2 that mediate homodimerization and interaction with general and melanocytic transcription factors. Eur J Biochem. 2000;267:6413-22 pubmed
  19. Arter J, Wegner M. Transcription factors Sox10 and Sox2 functionally interact with positive transcription elongation factor b in Schwann cells. J Neurochem. 2015;132:384-93 pubmed publisher
    ..Here, we show that Sox2 and Sox10, two Sox proteins important for Schwann cell development, also have the capability to activate transcriptional ..
  20. Lang D, Lu M, Huang L, Engleka K, Zhang M, Chu E, et al. Pax3 functions at a nodal point in melanocyte stem cell differentiation. Nature. 2005;433:884-7 pubmed
    ..Thus, a stem cell transcription factor can both determine cell fate and simultaneously maintain an undifferentiated state, leaving a cell poised to differentiate in response to external stimuli. ..
  21. Lane P, Liu H. Association of megacolon with a new dominant spotting gene (Dom) in the mouse. J Hered. 1984;75:435-9 pubmed
    ..6 +/- 1.6 units proximal to Ca. Hairy ears, Eh, a semidominant gene also on chr 15 is shown to have a suppressing effect on crossing over in this section of chr 15. ..
  22. Chen K, Zong L, Zhan Y, Wu X, Liu M, Jiang H. Genetic counseling for a three-generation Chinese family with Waardenburg syndrome type II associated with a rare SOX10 mutation. Int J Pediatr Otorhinolaryngol. 2015;79:745-8 pubmed publisher
    Waardenburg syndrome is clinically and genetically heterogeneous. The SOX10 mutation related with Waardenburg syndrome type II is rare in Chinese...
  23. Muth K, Piefke S, Weider M, Sock E, Hermans Borgmeyer I, Wegner M, et al. The Dual-specificity phosphatase Dusp15 is regulated by Sox10 and Myrf in Myelinating Oligodendrocytes. Glia. 2016;64:2120-2132 pubmed publisher
    ..The transcription factors Sox10 and Myrf are both central to this process and jointly regulate expression of myelin genes...
  24. Ito Y, Inoue N, Inoue Y, Nakamura S, Matsuda Y, Inagaki M, et al. Additive dominant effect of a SOX10 mutation underlies a complex phenotype of PCWH. Neurobiol Dis. 2015;80:1-14 pubmed publisher
    Distinct classes of SOX10 mutations result in peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease, collectively known as PCWH...
  25. Rehberg S, Lischka P, Glaser G, Stamminger T, Wegner M, Rosorius O. Sox10 is an active nucleocytoplasmic shuttle protein, and shuttling is crucial for Sox10-mediated transactivation. Mol Cell Biol. 2002;22:5826-34 pubmed
    b>Sox10 belongs to a family of transcription regulators characterized by a DNA-binding domain known as the HMG box...
  26. Kim J, Lo L, Dormand E, Anderson D. SOX10 maintains multipotency and inhibits neuronal differentiation of neural crest stem cells. Neuron. 2003;38:17-31 pubmed
    ..In neural crest stem cells (NCSCs), the HMG-box factor SOX10 preserves not only glial, but surprisingly, also neuronal potential from extinction by lineage commitment signals...
  27. Chen Z, Huang J, Liu Y, Dattilo L, Huh S, Ornitz D, et al. FGF signaling activates a Sox9-Sox10 pathway for the formation and branching morphogenesis of mouse ocular glands. Development. 2014;141:2691-701 pubmed publisher
    ..Sox9 regulates the expression of Sox10 to promote the formation of secretory acinar lobes in the lacrimal gland...
  28. Vogl M, Reiprich S, Küspert M, Kosian T, Schrewe H, Nave K, et al. Sox10 cooperates with the mediator subunit 12 during terminal differentiation of myelinating glia. J Neurosci. 2013;33:6679-90 pubmed publisher
    ..for terminal differentiation of myelinating glia, among them the high-mobility-group-domain-containing protein Sox10. To better understand how these factors exert their effects and shape glial expression programs, we identified and ..
  29. Mou Z, Tapper A, Gardner P. The armadillo repeat-containing protein, ARMCX3, physically and functionally interacts with the developmental regulatory factor Sox10. J Biol Chem. 2009;284:13629-40 pubmed publisher
    b>Sox10 is a member of the group E Sox transcription factor family and plays key roles in neural crest development and subsequent cellular differentiation...
  30. Leblanc S, Ward R, Svaren J. Neuropathy-associated Egr2 mutants disrupt cooperative activation of myelin protein zero by Egr2 and Sox10. Mol Cell Biol. 2007;27:3521-9 pubmed
    ..Egr2-responsive element within the Mpz first intron that also contains binding sites for the transcription factor Sox10. Furthermore, Egr2 activation through this element is impaired by mutation of the Sox10 binding sites...
  31. Hodonsky C, Kleinbrink E, Charney K, Prasad M, Bessling S, Jones E, et al. SOX10 regulates expression of the SH3-domain kinase binding protein 1 (Sh3kbp1) locus in Schwann cells via an alternative promoter. Mol Cell Neurosci. 2012;49:85-96 pubmed publisher
    The transcription factor SOX10 has essential roles in neural crest-derived cell populations, including myelinating Schwann cells-specialized glial cells responsible for ensheathing axons in the peripheral nervous system...
  32. Herbarth B, Pingault V, Bondurand N, Kuhlbrodt K, Hermans Borgmeyer I, Puliti A, et al. Mutation of the Sry-related Sox10 gene in Dominant megacolon, a mouse model for human Hirschsprung disease. Proc Natl Acad Sci U S A. 1998;95:5161-5 pubmed
    ..that the defect in the Dom mouse is caused by mutation of the gene encoding the Sry-related transcription factor Sox10. This assignment is based on (i) colocalization of the Sox10 gene with the Dom mutation on chromosome 15; (ii) ..