Gene Symbol: Sox10
Description: SRY (sex determining region Y)-box 10
Alias: Dom, Sox21, transcription factor SOX-10, SRY-box containing gene 10, dominant megacolon, transcription factor SOX-M
Species: mouse
Products:     Sox10

Top Publications

  1. Shakhova O, Zingg D, Schaefer S, Hari L, Civenni G, Blunschi J, et al. Sox10 promotes the formation and maintenance of giant congenital naevi and melanoma. Nat Cell Biol. 2012;14:882-90 pubmed publisher
    ..Here we describe a mouse model for giant congenital naevi and show that naevi and melanoma prominently express Sox10, a transcription factor crucial for the formation of melanocytes from the neural crest...
  2. Schreiner S, Cossais F, Fischer K, Scholz S, Bösl M, Holtmann B, et al. Hypomorphic Sox10 alleles reveal novel protein functions and unravel developmental differences in glial lineages. Development. 2007;134:3271-81 pubmed
    The transcription factor Sox10 regulates early neural crest development, specification of neural crest-derived lineages and terminal differentiation of oligodendrocytes in the central nervous system...
  3. 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 ..
  4. Seong I, Min H, Lee J, Yeo C, Kang D, Oh E, et al. Sox10 controls migration of B16F10 melanoma cells through multiple regulatory target genes. PLoS ONE. 2012;7:e31477 pubmed publisher
    ..b>Sox10, a transcription factor expressed in neural crest stem cells and a subset of progeny lineages, plays a key role in ..
  5. Barraud P, Seferiadis A, Tyson L, Zwart M, Szabo Rogers H, Ruhrberg C, et al. Neural crest origin of olfactory ensheathing glia. Proc Natl Acad Sci U S A. 2010;107:21040-5 pubmed publisher
  6. Finzsch M, Schreiner S, Kichko T, Reeh P, Tamm E, Bösl M, et al. Sox10 is required for Schwann cell identity and progression beyond the immature Schwann cell stage. J Cell Biol. 2010;189:701-12 pubmed publisher
    Mutations in the transcription factor SOX10 cause neurocristopathies, including Waardenburg-Hirschsprung syndrome and peripheral neuropathies in humans...
  7. Reiprich S, Kriesch J, Schreiner S, Wegner M. Activation of Krox20 gene expression by Sox10 in myelinating Schwann cells. J Neurochem. 2010;112:744-54 pubmed publisher
    The high-mobility group domain transcription factor Sox10 is believed to influence myelination in Schwann cells by directly activating myelin genes and by inducing Krox20 as a pivotal regulator of peripheral myelination...
  8. Weider M, K spert M, Bischof M, Vogl M, Hornig J, Loy K, et al. Chromatin-remodeling factor Brg1 is required for Schwann cell differentiation and myelination. Dev Cell. 2012;23:193-201 pubmed publisher
    ..How this is linked to chromatin changes is not well understood. Here we show that the glial transcription factor Sox10 functions in Schwann cells by recruiting Brg1-containing chromatin-remodeling complexes via Baf60a to regulatory ..
  9. Schwarz Q, Maden C, Vieira J, Ruhrberg C. Neuropilin 1 signaling guides neural crest cells to coordinate pathway choice with cell specification. Proc Natl Acad Sci U S A. 2009;106:6164-9 pubmed publisher
    ..We conclude that NRP1 signaling coordinates pathway choice with NCC fate and therefore confines neuronal differentiation to appropriate locations. ..

More Information


  1. 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 ..
  2. Schwarz Q, Vieira J, Howard B, Eickholt B, Ruhrberg C. Neuropilin 1 and 2 control cranial gangliogenesis and axon guidance through neural crest cells. Development. 2008;135:1605-13 pubmed publisher
    ..We conclude that neuropilins play multiple roles in the sensory nervous system by directing cranial neural crest cells, positioning sensory neurons and organising their axonal projections. ..
  3. 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
    The spontaneous mouse mutant Dominant megacolon (Dom) is a valuable model for the study of human congenital megacolon (Hirschsprung disease)...
  4. Potterf S, Furumura M, Dunn K, Arnheiter H, Pavan W. Transcription factor hierarchy in Waardenburg syndrome: regulation of MITF expression by SOX10 and PAX3. Hum Genet. 2000;107:1-6 pubmed
    ..melanocyte deficiency caused by mutations in either one of three transcription factors: MITF, PAX3, and SOX10. However, the hierarchical relationship of these transcription factors is largely unknown...
  5. Harris M, Buac K, Shakhova O, Hakami R, Wegner M, Sommer L, et al. A dual role for SOX10 in the maintenance of the postnatal melanocyte lineage and the differentiation of melanocyte stem cell progenitors. PLoS Genet. 2013;9:e1003644 pubmed publisher
    During embryogenesis, the transcription factor, Sox10, drives the survival and differentiation of the melanocyte lineage. However, the role that Sox10 plays in postnatal melanocytes is not established...
  6. Breuskin I, Bodson M, Thelen N, Thiry M, Borgs L, Nguyen L, et al. Glial but not neuronal development in the cochleo-vestibular ganglion requires Sox10. J Neurochem. 2010;114:1827-39 pubmed publisher
    ..Here, we report that Sox10, a high-mobility group DNA-binding domain transcription factor that is required for the proper development of ..
  7. Pattyn A, Morin X, Cremer H, Goridis C, Brunet J. The homeobox gene Phox2b is essential for the development of autonomic neural crest derivatives. Nature. 1999;399:366-70 pubmed
  8. Bremer M, Fröb F, Kichko T, Reeh P, Tamm E, Suter U, et al. Sox10 is required for Schwann-cell homeostasis and myelin maintenance in the adult peripheral nerve. Glia. 2011;59:1022-32 pubmed publisher
    The transcription factor Sox10 functions during multiple consecutive stages of Schwann-cell development in the peripheral nervous system (PNS)...
  9. Schlierf B, Werner T, Glaser G, Wegner M. Expression of connexin47 in oligodendrocytes is regulated by the Sox10 transcription factor. J Mol Biol. 2006;361:11-21 pubmed
    ..Here, we identify group E Sox proteins, in particular Sox10, as essential transcriptional regulators of both connexins...
  10. Deal K, Cantrell V, Chandler R, Saunders T, Mortlock D, Southard Smith E. Distant regulatory elements in a Sox10-beta GEO BAC transgene are required for expression of Sox10 in the enteric nervous system and other neural crest-derived tissues. Dev Dyn. 2006;235:1413-32 pubmed
    b>Sox10 is an essential transcription factor required for development of neural crest-derived melanocytes, peripheral glia, and enteric ganglia...
  11. John N, Cinelli P, Wegner M, Sommer L. Transforming growth factor ?-mediated Sox10 suppression controls mesenchymal progenitor generation in neural crest stem cells. Stem Cells. 2011;29:689-99 pubmed publisher
    ..Here, we show that transforming growth factor ? (TGF?)-mediated suppression of the NCSC transcription factor Sox10 induces a switch in neural to mesenchymal potential in NCSCs...
  12. 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. ..
  13. Pusch C, Hustert E, Pfeifer D, Sudbeck P, Kist R, Roe B, et al. The SOX10/Sox10 gene from human and mouse: sequence, expression, and transactivation by the encoded HMG domain transcription factor. Hum Genet. 1998;103:115-23 pubmed
    ..We have cloned and sequenced the SOX10 and Sox10 genes from human and mouse, respectively...
  14. Taylor M, Yeager K, Morrison S. Physiological Notch signaling promotes gliogenesis in the developing peripheral and central nervous systems. Development. 2007;134:2435-47 pubmed
    ..These results demonstrate that physiological Notch signaling is required for gliogenesis in vivo, independent of the role of Notch in the maintenance of undifferentiated neural progenitors. ..
  15. Küspert M, Weider M, Müller J, Hermans Borgmeyer I, Meijer D, Wegner M. Desert hedgehog links transcription factor Sox10 to perineurial development. J Neurosci. 2012;32:5472-80 pubmed publisher
    ..They develop along nerves during embryogenesis and rely on the HMG domain containing Sox10 transcription factor for specification, lineage progression, and terminal differentiation...
  16. Zhu Y, Jia Y, Hu L, Qi C, Prasad M, McCallion A, et al. Peroxisome-proliferator-activated receptor-binding protein (PBP) is essential for the growth of active Notch4-immortalized mammary epithelial cells by activating SOX10 expression. Biochem J. 2009;425:435-44 pubmed publisher
    ..Loss of PBP expression was shown to completely ablate the expression of SOX10 [Sry-related HMG (high-mobility group) box gene 10]...
  17. Coate T, Raft S, Zhao X, Ryan A, Crenshaw E, Kelley M. Otic mesenchyme cells regulate spiral ganglion axon fasciculation through a Pou3f4/EphA4 signaling pathway. Neuron. 2012;73:49-63 pubmed publisher
    ..These results indicate a model whereby Pou3f4 in the otic mesenchyme establishes an Eph/ephrin-mediated fasciculation signal that promotes inner radial bundle formation. ..
  18. Huettl R, Soellner H, Bianchi E, Novitch B, Huber A. Npn-1 contributes to axon-axon interactions that differentially control sensory and motor innervation of the limb. PLoS Biol. 2011;9:e1001020 pubmed publisher
    ..Thus, motor and sensory axons are mutually dependent on each other for the generation of their trajectories and interact in part through Npn-1-mediated fasciculation before and within the plexus region of the limbs...
  19. Stolt C, Lommes P, Sock E, Chaboissier M, Schedl A, Wegner M. The Sox9 transcription factor determines glial fate choice in the developing spinal cord. Genes Dev. 2003;17:1677-89 pubmed
    ..progenitor numbers recovered at later stages of development, probably owing to compensatory actions of the related Sox10 and Sox8, both of which overlap with Sox9 in the oligodendrocyte lineage...
  20. Cossais F, Sock E, Hornig J, Schreiner S, Kellerer S, Bösl M, et al. Replacement of mouse Sox10 by the Drosophila ortholog Sox100B provides evidence for co-option of SoxE proteins into vertebrate-specific gene-regulatory networks through altered expression. Dev Biol. 2010;341:267-81 pubmed publisher
    ..regulatory networks, of which the SoxE-type high-mobility-group domain transcription factors Sox8, Sox9 and Sox10 are essential components...
  21. Stolt C, Schlierf A, Lommes P, Hillgärtner S, Werner T, Kosian T, et al. SoxD proteins influence multiple stages of oligodendrocyte development and modulate SoxE protein function. Dev Cell. 2006;11:697-709 pubmed
    ..Sox5 and Sox6 have opposite functions than the group E Sox proteins Sox9 and Sox10, which promote oligodendrocyte specification and terminal differentiation...
  22. Bondurand N, Sham M. The role of SOX10 during enteric nervous system development. Dev Biol. 2013;382:330-43 pubmed publisher
    The SOX10 transcription factor is a characteristic marker for migratory multipotent neural crest (NC) progenitors as well as several of their differentiated derivatives...
  23. Trokovic N, Trokovic R, Partanen J. Fibroblast growth factor signalling and regional specification of the pharyngeal ectoderm. Int J Dev Biol. 2005;49:797-805 pubmed
    ..Our results suggest that Fgfr1 is important for localized signalling in the pharyngeal ectoderm and consequently for normal tissue interactions in the developing second branchial arch...
  24. 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...
  25. Britsch S, Goerich D, Riethmacher D, Peirano R, Rossner M, Nave K, et al. The transcription factor Sox10 is a key regulator of peripheral glial development. Genes Dev. 2001;15:66-78 pubmed
    ..We show here that the transcription factor Sox10 is a key regulator in differentiation of peripheral glial cells...
  26. Hari L, Miescher I, Shakhova O, Suter U, Chin L, Taketo M, et al. Temporal control of neural crest lineage generation by Wnt/?-catenin signaling. Development. 2012;139:2107-17 pubmed publisher
    ..Thus, neural crest cells appear to be multipotent in vivo both before and after emigration from the neural tube but adapt their response to extracellular signals in a temporally controlled manner. ..
  27. 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
    ..are reminiscent of the white coat-spotting and aganglionic megacolon displayed by the mouse mutants Dom (Dominant megacolon), piebald-lethal (sl) and lethal spotting (ls)...
  28. Cossais F, Wahlbuhl M, Kriesch J, Wegner M. SOX10 structure-function analysis in the chicken neural tube reveals important insights into its role in human neurocristopathies. Hum Mol Genet. 2010;19:2409-20 pubmed publisher
    The HMG-domain containing transcription factor Sox10 is essential for neural crest (NC) development and for oligodendrocyte differentiation...
  29. Lavery R, Lardenois A, Ranc Jianmotamedi F, Pauper E, Gregoire E, Vigier C, et al. XY Sox9 embryonic loss-of-function mouse mutants show complete sex reversal and produce partially fertile XY oocytes. Dev Biol. 2011;354:111-22 pubmed publisher
    ..Taken together, we found that XY Sf1:Cre(Tg/+); Sox9(flox/flox) females are capable of producing viable offspring albeit at a reduced level. ..
  30. Southard Smith E, Angrist M, Ellison J, Agarwala R, Baxevanis A, Chakravarti A, et al. The Sox10(Dom) mouse: modeling the genetic variation of Waardenburg-Shah (WS4) syndrome. Genome Res. 1999;9:215-25 pubmed
    ..b>Sox10(Dom)/+ mice exhibit variability of aganglionosis and hypopigmentation influenced by genetic background similar to ..
  31. Paratore C, Eichenberger C, Suter U, Sommer L. Sox10 haploinsufficiency affects maintenance of progenitor cells in a mouse model of Hirschsprung disease. Hum Mol Genet. 2002;11:3075-85 pubmed
    ..Several genes involved in the disease have been identified. In particular, haploinsufficiency of SOX10, which encodes a transcription factor, results in megacolon, often in combination with other disorders...
  32. Srinivasan R, Sun G, Keles S, Jones E, Jang S, Krueger C, et al. Genome-wide analysis of EGR2/SOX10 binding in myelinating peripheral nerve. Nucleic Acids Res. 2012;40:6449-60 pubmed publisher
    ..Two critical determinants of myelination are SOX10 and EGR2/KROX20...
  33. Wahlbuhl M, Reiprich S, Vogl M, Bösl M, Wegner M. Transcription factor Sox10 orchestrates activity of a neural crest-specific enhancer in the vicinity of its gene. Nucleic Acids Res. 2012;40:88-101 pubmed publisher
    The Sox10 transcription factor is a central regulator of vertebrate neural crest and nervous system development. Its expression is likely controlled by multiple enhancer elements, among them U3 (alternatively known as MCS4)...
  34. Tekki Kessaris N, Woodruff R, Hall A, Gaffield W, Kimura S, Stiles C, et al. Hedgehog-dependent oligodendrocyte lineage specification in the telencephalon. Development. 2001;128:2545-54 pubmed
    ..that co-expresses mRNA encoding SHH, its receptor PTC1 (PTCH) and the transcription factors OLIG1, OLIG2 and SOX10. Pdgfra-positive progenitors later spread through the forebrain into areas where Shh is not expressed, including ..
  35. Roffers Agarwal J, Gammill L. Neuropilin receptors guide distinct phases of sensory and motor neuronal segmentation. Development. 2009;136:1879-88 pubmed publisher
    ..These data show that Nrp2 and Nrp1 work together to produce segmentation of sensory and motor nerves, and that dorsal peripheral nervous system metamerism is generated in a stepwise, Nrp-dependent process. ..
  36. Motohashi T, Yamanaka K, Chiba K, Miyajima K, Aoki H, Hirobe T, et al. Neural crest cells retain their capability for multipotential differentiation even after lineage-restricted stages. Dev Dyn. 2011;240:1681-93 pubmed publisher
    ..We analyzed the differentiation of early-stage NC-like cells derived from Sox10-IRES-Venus ES cells, where the expression of Sox10 can be visualized with a fluorescent protein...
  37. Gammill L, Roffers Agarwal J. Division of labor during trunk neural crest development. Dev Biol. 2010;344:555-65 pubmed publisher
    ..Finally, we compare cranial and trunk neural crest development in order to highlight common themes. ..
  38. Hou L, Arnheiter H, Pavan W. Interspecies difference in the regulation of melanocyte development by SOX10 and MITF. Proc Natl Acad Sci U S A. 2006;103:9081-5 pubmed
    ..In both zebrafish and mice, one transcription factor, SOX10, controls the expression of another, MITF (microphthalmia-associated transcription factor), which in turn ..
  39. 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...
  40. Corpening J, Deal K, Cantrell V, Skelton S, Buehler D, Southard Smith E. Isolation and live imaging of enteric progenitors based on Sox10-Histone2BVenus transgene expression. Genesis. 2011;49:599-618 pubmed publisher
    ..viewing, we generated a mouse BAC transgenic line that drives a Histone2BVenus (H2BVenus) reporter from Sox10 regulatory regions...
  41. 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...
  42. Wegner M. From head to toes: the multiple facets of Sox proteins. Nucleic Acids Res. 1999;27:1409-20 pubmed
    ..Sox proteins perform their function in a complex interplay with other transcription factors in a manner highly dependent on cell type and promoter context. They exhibit a remarkable crosstalk and functional redundancy among each other. ..
  43. Schwarz Q, Maden C, Davidson K, Ruhrberg C. Neuropilin-mediated neural crest cell guidance is essential to organise sensory neurons into segmented dorsal root ganglia. Development. 2009;136:1785-9 pubmed publisher
    ..By contrast, the combined loss of both guidance pathways leads to ectopic invasion of the intersomitic furrows and posterior sclerotome halves, disrupting metameric NCC streaming and DRG segmentation. ..
  44. Vallstedt A, Klos J, Ericson J. Multiple dorsoventral origins of oligodendrocyte generation in the spinal cord and hindbrain. Neuron. 2005;45:55-67 pubmed
    ..Based on these findings, we propose that oligodendrocytes derive from several distinct positional origins and that the activation of Olig1/2 at different positions is controlled by distinct genetic programs. ..
  45. Young H, Bergner A, Muller T. Acquisition of neuronal and glial markers by neural crest-derived cells in the mouse intestine. J Comp Neurol. 2003;456:1-11 pubmed
    ..Previous studies have shown that undifferentiated enteric crest-derived cells are Phox2b(+)/Ret(+)/p75(+)/Sox10(+), and at embryonic day (E) 10...
  46. De Bellard M, Ching W, Gossler A, Bronner Fraser M. Disruption of segmental neural crest migration and ephrin expression in delta-1 null mice. Dev Biol. 2002;249:121-30 pubmed
    ..Interestingly, absence of Delta-1 leads to diminution of both neurons and glia in peripheral ganglia, suggesting a general depletion of the ganglion precursor pool in mutant mice. ..
  47. Cronin J, Watkins Chow D, Incao A, Hasskamp J, Schönewolf N, Aoude L, et al. SOX10 ablation arrests cell cycle, induces senescence, and suppresses melanomagenesis. Cancer Res. 2013;73:5709-18 pubmed publisher
    The transcription factor SOX10 is essential for survival and proper differentiation of neural crest cell lineages, where it plays an important role in the generation and maintenance of melanocytes...
  48. Potterf S, Mollaaghababa R, Hou L, Southard Smith E, Hornyak T, Arnheiter H, et al. Analysis of SOX10 function in neural crest-derived melanocyte development: SOX10-dependent transcriptional control of dopachrome tautomerase. Dev Biol. 2001;237:245-57 pubmed
    b>SOX10 is a high-mobility-group transcription factor that plays a critical role in the development of neural crest-derived melanocytes. At E11...
  49. Lee K, Nam S, Cho E, Seong I, Limb J, Lee S, et al. Identification of direct regulatory targets of the transcription factor Sox10 based on function and conservation. BMC Genomics. 2008;9:408 pubmed publisher
    b>Sox10, a member of the Sry-related HMG-Box gene family, is a critical transcription factor for several important cell lineages, most notably the neural crest stem cells and the derivative peripheral glial cells and melanocytes...
  50. Liu A, Li J, Marin Husstege M, Kageyama R, Fan Y, Gelinas C, et al. A molecular insight of Hes5-dependent inhibition of myelin gene expression: old partners and new players. EMBO J. 2006;25:4833-42 pubmed
    ..oligodendrocyte lineage include the regulation of feedback loops with the cell-specific transcriptional activator Sox10. In progenitors with low levels of Sox10, Hes5 further decreases the bioavailability of this protein by ..
  51. Anderson R, Stewart A, Young H. Phenotypes of neural-crest-derived cells in vagal and sacral pathways. Cell Tissue Res. 2006;323:11-25 pubmed
    ..To examine the phenotype of neural-crest-derived cells in vagal and sacral pathways, we used antisera to Sox10, p75, Phox2b, and Hu, and transgenic mice in which the expression of green fluorescent protein was under the ..
  52. Gammill L, Gonzalez C, Gu C, Bronner Fraser M. Guidance of trunk neural crest migration requires neuropilin 2/semaphorin 3F signaling. Development. 2006;133:99-106 pubmed
    ..Interestingly, neural crest cells still condense into segmentally arranged dorsal root ganglia in Npn2 nulls, suggesting that segmental neural crest migration and segmentation of the peripheral nervous system are separable processes. ..
  53. Freyer L, Aggarwal V, Morrow B. Dual embryonic origin of the mammalian otic vesicle forming the inner ear. Development. 2011;138:5403-14 pubmed publisher
    ..This study defines a dual cellular origin of the inner ear from sensory placode ectoderm and NECs, and changes the current paradigm of inner ear neurosensory development. ..
  54. 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...
  55. Fröb F, Bremer M, Finzsch M, Kichko T, Reeh P, Tamm E, et al. Establishment of myelinating Schwann cells and barrier integrity between central and peripheral nervous systems depend on Sox10. Glia. 2012;60:806-19 pubmed publisher
    The transcription factor Sox10 is expressed throughout Schwann cell development and has already been shown to be essential for specification and for the identity and further development of immature Schwann cells...
  56. Liu Z, Hu X, Cai J, Liu B, Peng X, Wegner M, et al. Induction of oligodendrocyte differentiation by Olig2 and Sox10: evidence for reciprocal interactions and dosage-dependent mechanisms. Dev Biol. 2007;302:683-93 pubmed
    ..In the developing mouse spinal cord, expression of Olig2, Sox10 and Nkx2.2 is sequentially up-regulated in cells of oligodendrocyte lineage...
  57. 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 ..
  58. Paratore C, Goerich D, Suter U, Wegner M, Sommer L. Survival and glial fate acquisition of neural crest cells are regulated by an interplay between the transcription factor Sox10 and extrinsic combinatorial signaling. Development. 2001;128:3949-61 pubmed
    The transcription factor Sox10 is required for proper development of various neural crest-derived cell types...
  59. Potzner M, Tsarovina K, BINDER E, Penzo Méndez A, Lefebvre V, Rohrer H, et al. Sequential requirement of Sox4 and Sox11 during development of the sympathetic nervous system. Development. 2010;137:775-84 pubmed publisher
    ..As a consequence, sympathetic ganglia were rudimentary in the adult and sympathetic innervation of target tissues was impaired leading to severe dysautonomia. ..
  60. Tsarovina K, Pattyn A, Stubbusch J, Muller F, van der Wees J, Schneider C, et al. Essential role of Gata transcription factors in sympathetic neuron development. Development. 2004;131:4775-86 pubmed
    ..The present data establish Gata2 and Gata3 in the chick and mouse, respectively, as essential members of the transcription factor network controlling sympathetic neuron development. ..
  61. Werner T, Hammer A, Wahlbuhl M, Bösl M, Wegner M. Multiple conserved regulatory elements with overlapping functions determine Sox10 expression in mouse embryogenesis. Nucleic Acids Res. 2007;35:6526-38 pubmed
    Expression and function of the transcription factor Sox10 is predominant in neural crest cells, its derivatives and in oligodendrocytes...
  62. Cai J, Qi Y, Hu X, Tan M, Liu Z, Zhang J, et al. Generation of oligodendrocyte precursor cells from mouse dorsal spinal cord independent of Nkx6 regulation and Shh signaling. Neuron. 2005;45:41-53 pubmed
    ..In addition, we provide genetic evidence that oligodendrogenesis can occur in the absence of hedgehog receptor Smoothened, which is essential for all hedgehog signaling. ..
  63. Yu Y, Chen Y, Kim B, Wang H, Zhao C, He X, et al. Olig2 targets chromatin remodelers to enhancers to initiate oligodendrocyte differentiation. Cell. 2013;152:248-61 pubmed publisher
  64. Machold R, Hayashi S, Rutlin M, Muzumdar M, Nery S, Corbin J, et al. Sonic hedgehog is required for progenitor cell maintenance in telencephalic stem cell niches. Neuron. 2003;39:937-50 pubmed
    ..These results suggest that hedgehog signaling is required to maintain progenitor cells in the postnatal telencephalon. ..
  65. Peirano R, Goerich D, Riethmacher D, Wegner M. Protein zero gene expression is regulated by the glial transcription factor Sox10. Mol Cell Biol. 2000;20:3198-209 pubmed
    ..of the peripheral nervous system, is controlled in its expression by the high-mobility-group domain protein Sox10 both in tissue culture and in vivo...
  66. Polanco J, Wilhelm D, Davidson T, Knight D, Koopman P. Sox10 gain-of-function causes XX sex reversal in mice: implications for human 22q-linked disorders of sex development. Hum Mol Genet. 2010;19:506-16 pubmed publisher
    ..Here, we show that transgenic expression of Sox10, a close relative of Sox9, in gonads of XX mice resulted in development of testes and male physiology...
  67. Antonellis A, Huynh J, Lee Lin S, Vinton R, Renaud G, Loftus S, et al. Identification of neural crest and glial enhancers at the mouse Sox10 locus through transgenesis in zebrafish. PLoS Genet. 2008;4:e1000174 pubmed publisher
    b>Sox10 is a dynamically regulated transcription factor gene that is essential for the development of neural crest-derived and oligodendroglial populations...
  68. 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...
  69. Antonellis A, Bennett W, Menheniott T, Prasad A, Lee Lin S, Green E, et al. Deletion of long-range sequences at Sox10 compromises developmental expression in a mouse model of Waardenburg-Shah (WS4) syndrome. Hum Mol Genet. 2006;15:259-71 pubmed
    The transcription factor SOX10 is mutated in the human neurocristopathy Waardenburg-Shah syndrome (WS4), which is characterized by enteric aganglionosis and pigmentation defects...
  70. Compagnucci C, Fish J, Schwark M, Tarabykin V, Depew M. Pax6 regulates craniofacial form through its control of an essential cephalic ectodermal patterning center. Genesis. 2011;49:307-25 pubmed publisher
    ..Pax6 therefore regulates craniofacial form, at stages when CNC has just arrived in the frontonasal region, through its control of surface cephalic ectodermal competence to form an essential craniofacial patterning center. ..
  71. Mollaaghababa R, Pavan W. The importance of having your SOX on: role of SOX10 in the development of neural crest-derived melanocytes and glia. Oncogene. 2003;22:3024-34 pubmed
    ..Disruption of neural crest development in the Dominant megacolon (Dom) mice is associated with a Sox10 mutation...
  72. Okamura Y, Saga Y. Notch signaling is required for the maintenance of enteric neural crest progenitors. Development. 2008;135:3555-65 pubmed publisher
    ..We found that Sox10 expression, which is normally maintained in ENCC progenitors, was decreased in Pofut1-null ENCCs...
  73. Stolt C, Lommes P, Hillgärtner S, Wegner M. The transcription factor Sox5 modulates Sox10 function during melanocyte development. Nucleic Acids Res. 2008;36:5427-40 pubmed publisher
    ..Loss of Sox5, however, partially rescued the strongly reduced melanoblast generation and marker gene expression in Sox10 heterozygous mice arguing that Sox5 functions in the melanocyte lineage by modulating Sox10 activity...
  74. Wegener A, Deboux C, Bachelin C, Frah M, Kerninon C, Seilhean D, et al. Gain of Olig2 function in oligodendrocyte progenitors promotes remyelination. Brain. 2015;138:120-35 pubmed publisher
    ..To decipher Olig2 functions, we generated transgenic mice (TetOlig2:Sox10(rtTA/+)) overexpressing Olig2 in Sox10(+) oligodendroglial cells in a doxycycline inducible manner...
  75. Liu R, Cai J, Hu X, Tan M, Qi Y, German M, et al. Region-specific and stage-dependent regulation of Olig gene expression and oligodendrogenesis by Nkx6.1 homeodomain transcription factor. Development. 2003;130:6221-31 pubmed
    ..In the hindbrain, unlike in the spinal cord, Olig1 and Olig2 can be expressed both inside and outside the Nkx6.1-expressing domains and oligodendrogenesis in this region is not dependent on Nkx6.1 activity. ..
  76. Finzsch M, Stolt C, Lommes P, Wegner M. Sox9 and Sox10 influence survival and migration of oligodendrocyte precursors in the spinal cord by regulating PDGF receptor alpha expression. Development. 2008;135:637-46 pubmed publisher
    ..system requires the Sox9 transcription factor, whereas terminal differentiation depends on the closely related Sox10. Between specification and terminal differentiation, Sox9 and Sox10 are co-expressed in oligodendrocyte precursors ..
  77. Bogani D, Warr N, Elms P, Davies J, Tymowska Lalanne Z, Goldsworthy M, et al. New semidominant mutations that affect mouse development. Genesis. 2004;40:109-117 pubmed
    ..In one coat spotting mutant the homozygous condition is lethal before neural crest cell production commences. The mutated genes often function in processes additional to those alluded to by the heterozygous phenotype. ..
  78. Sun T, Echelard Y, Lu R, Yuk D, Kaing S, Stiles C, et al. Olig bHLH proteins interact with homeodomain proteins to regulate cell fate acquisition in progenitors of the ventral neural tube. Curr Biol. 2001;11:1413-20 pubmed
    ..Moreover, coexpression of Olig2 with Nkx2.2 in the chick neural tube generated cells expressing Sox10, a marker of oligodendroglial precursors. Colocalization of Olig and Nkx2...
  79. Forni P, Taylor Burds C, Melvin V, Williams T, Williams T, Wray S. Neural crest and ectodermal cells intermix in the nasal placode to give rise to GnRH-1 neurons, sensory neurons, and olfactory ensheathing cells. J Neurosci. 2011;31:6915-27 pubmed publisher
    ..Furthermore, the results indicate that certain conditions that impact olfaction and sexual development, such as Kallmann syndrome, may be in part neurocristopathies...
  80. Inoue T, Hatayama M, Tohmonda T, Itohara S, Aruga J, Mikoshiba K. Mouse Zic5 deficiency results in neural tube defects and hypoplasia of cephalic neural crest derivatives. Dev Biol. 2004;270:146-62 pubmed
    ..Based on both their similar expression pattern in mouse embryos and the malformations observed in Zic5-deficient mutant mice, human ZIC5 might be involved in the deletion syndrome. ..
  81. Kamachi Y, Uchikawa M, Kondoh H. Pairing SOX off: with partners in the regulation of embryonic development. Trends Genet. 2000;16:182-7 pubmed
  82. Dennis J, Kurosaka H, Iulianella A, Pace J, Thomas N, Beckham S, et al. Mutations in Hedgehog acyltransferase (Hhat) perturb Hedgehog signaling, resulting in severe acrania-holoprosencephaly-agnathia craniofacial defects. PLoS Genet. 2012;8:e1002927 pubmed publisher
    ..Future genetic studies should include HHAT as a potential candidate in the etiology and pathogenesis of HPE and its associated disorders. ..
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    ..Moreover, this region contains conserved binding sites not only for Egr2 but also for Sox10, which is also required for Schwann cell development...