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Gene Review

Shox2  -  short stature homeobox 2

Mus musculus

Synonyms: 6330543G17Rik, Homeobox protein Og12X, OG-12, OG12, Og12x, ...
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High impact information on Shox2

  • In rat and mouse embryos, nonneuronal structures around the oral cavity and in hip and shoulder regions also expressed the Prx3 gene [1].
  • In the adult rat brain, Prx3 gene expression was restricted to thalamic, tectal, and brainstem structures that include relay nuclei of the visual and auditory systems as well as other ascending systems conveying somatosensory information [1].
  • Moreover, this paper reports a complex and monophasic pattern of Ol-Prx 3 expression in the central nervous system, which differs markedly from the patterns reported for the PRX genes, Prx 3 excluded: this gene begins to be expressed in a variety of central nervous system territories at late neurula stage [2].
  • SHOX is, however, closely related to the SHOX2 homeobox gene on 3q, which has a murine counterpart, Og12x [3].
  • OG12X was mapped to human chromosome 3q22-26 and murine Og12x to the syntenic region on mouse chromosome 3 [4].

Anatomical context of Shox2

  • By contrast, the Shox2-/- mice exhibit incomplete clefts in the anterior presumptive hard palate with an intact posterior palate [5].
  • In addition, the anterior palatal epithelium has the unique ability to induce Shox2 expression in the anterior mesenchyme in vivo and the posterior mesenchyme in vitro [5].
  • Tissue recombination and bead implantation experiments revealed that signals from the anterior palatal epithelium are responsible for the restricted mesenchymal Shox2 expression [6].
  • In situ hybridization on mouse embryos ranging from 9 to 16 days post-coitum localized murine Og12x mRNA in the heart, otic region, maxillary and mandibular components of the first branchial arch, nasal processes, eyelid, midbrain, medulla oblongata, limbs, dorsal root ganglia and genital tubercle [4].
  • Using quantitative real-time PCR (Q-PCR), we show that Prx3 is also downregulated in spinal motor neurons from patients with both sporadic (sMND) and SOD1-related fMND [7].

Analytical, diagnostic and therapeutic context of Shox2


  1. Homeobox gene Prx3 expression in rodent brain and extraneural tissues. van Schaick, H.S., Smidt, M.P., Rovescalli, A.C., Luijten, M., van der Kleij, A.A., Asoh, S., Kozak, C.A., Nirenberg, M., Burbach, J.P. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  2. Ol-Prx 3, a member of an additional class of homeobox genes, is unimodally expressed in several domains of the developing and adult central nervous system of the medaka (Oryzias latipes). Joly, J.S., Bourrat, F., Nguyen, V., Chourrout, D. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  3. The short stature homeobox gene SHOX is involved in skeletal abnormalities in Turner syndrome. Clement-Jones, M., Schiller, S., Rao, E., Blaschke, R.J., Zuniga, A., Zeller, R., Robson, S.C., Binder, G., Glass, I., Strachan, T., Lindsay, S., Rappold, G.A. Hum. Mol. Genet. (2000) [Pubmed]
  4. A new human homeobox gene OGI2X is a member of the most conserved homeobox gene family and is expressed during heart development in mouse. Semina, E.V., Reiter, R.S., Murray, J.C. Hum. Mol. Genet. (1998) [Pubmed]
  5. Regional regulation of palatal growth and patterning along the anterior-posterior axis in mice. Hilliard, S.A., Yu, L., Gu, S., Zhang, Z., Chen, Y.P. J. Anat. (2005) [Pubmed]
  6. Shox2-deficient mice exhibit a rare type of incomplete clefting of the secondary palate. Yu, L., Gu, S., Alappat, S., Song, Y., Yan, M., Zhang, X., Zhang, G., Jiang, Y., Zhang, Z., Zhang, Y., Chen, Y. Development (2005) [Pubmed]
  7. Impairment of mitochondrial anti-oxidant defence in SOD1-related motor neuron injury and amelioration by ebselen. Wood-Allum, C.A., Barber, S.C., Kirby, J., Heath, P., Holden, H., Mead, R., Higginbottom, A., Allen, S., Beaujeux, T., Alexson, S.E., Ince, P.G., Shaw, P.J. Brain (2006) [Pubmed]
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