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Gene Review

SOX3  -  SRY (sex determining region Y)-box 3

Gallus gallus

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Disease relevance of SOX3


High impact information on SOX3

  • The oncogenicity of Sox3 is correlated with nuclear localization and transcriptional regulatory activity; mutants containing deletions in the HMG box or the transactivation domain fail to induce foci of transformation [2].
  • Ectopic expression of Sox3 causes oncogenic transformation of chicken embryo fibroblasts (CEFs) [2].
  • After tight association of the optic vesicle (around stage 10-11, 40 hours after egg incubation), expression of Sox2 and Sox3 is activated in the vesicle-facing ectoderm at stage 12 (44 hours) [3].
  • Different combinations of signals are responsible for different aspects of this early transient induction: FGF initiates expression of Sox3 and ERNI, retinoic acid can induce Cyp26A1 and only a combination of low levels of FGF8 together with Wnt- and BMP-antagonists can induce Otx2 [4].
  • SOX3 expression was observed prior to clear SOX9 expression and was up-regulated in both sexes at the onset of gonadal sex differentiation (but declined later in development) [5].

Anatomical context of SOX3

  • These data suggest that deregulation of SOX3 target genes and inappropriate canonical Wnt signaling in central nervous system (CNS) progenitors may also contribute to dysfunction of the hypothalamic-pituitary axis in XH patients [1].

Other interactions of SOX3

  • Dynamic expression of chicken Sox2 and Sox3 genes in ectoderm induced to form neural tissue [6].
  • Semi-quantitative RT-PCE analysis of the expression of several transcription factors indicated that Sox1 and Sox3 were upregulated in parallel with delta1-crystallin mRNA in form-deprived eyes [7].


  1. Polyalanine expansion mutations in the X-linked hypopituitarism gene SOX3 result in aggresome formation and impaired transactivation. Wong, J., Farlie, P., Holbert, S., Lockhart, P., Thomas, P.Q. Front. Biosci. (2007) [Pubmed]
  2. The oncogenic potential of the high mobility group box protein Sox3. Xia, Y., Papalopulu, N., Vogt, P.K., Li, J. Cancer Res. (2000) [Pubmed]
  3. Involvement of Sox1, 2 and 3 in the early and subsequent molecular events of lens induction. Kamachi, Y., Uchikawa, M., Collignon, J., Lovell-Badge, R., Kondoh, H. Development (1998) [Pubmed]
  4. A role for the hypoblast (AVE) in the initiation of neural induction, independent of its ability to position the primitive streak. Albazerchi, A., Stern, C.D. Dev. Biol. (2007) [Pubmed]
  5. Gene expression during gonadogenesis in the chicken embryo. Smith, C.A., Smith, M.J., Sinclair, A.H. Gene (1999) [Pubmed]
  6. Dynamic expression of chicken Sox2 and Sox3 genes in ectoderm induced to form neural tissue. Rex, M., Orme, A., Uwanogho, D., Tointon, K., Wigmore, P.M., Sharpe, P.T., Scotting, P.J. Dev. Dyn. (1997) [Pubmed]
  7. Up-regulation of crystallin mRNAs in form-deprived chick eyes. Ishibashi, K., Fujii, S., Escaño, M.F., Sekiya, Y., Yamamoto, M. Exp. Eye Res. (2000) [Pubmed]
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