The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Dlx2  -  distal-less homeobox 2

Mus musculus

Synonyms: AW121999, DII A, Dlx-2, Homeobox protein DLX-2, Homeobox protein TES-1, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Dlx2

  • Dlx1 and Dlx2 expression was seen only in nonneuronal cells of the cochleovestibular ganglion and nerves from stage 21 to stage 32 [1].
  • Recent evidence indicates that in utero injection of the E9.5 mouse forebrain with retroviruses encoding wild-type Shh induces the ectopic expression of Dlx2 and severe deformities in the brain [2].

High impact information on Dlx2

  • The Evf-2 noncoding RNA is transcribed from the Dlx-5/6 ultraconserved region and functions as a Dlx-2 transcriptional coactivator [3].
  • In previous experiments, we showed that Sonic hedgehog treatment of forebrain neural explants results in the activation of Dlx-2 and the novel noncoding RNA (ncRNA), Evf-1 [3].
  • Evf-2 specifically cooperates with Dlx-2 to increase the transcriptional activity of the Dlx-5/6 enhancer in a target and homeodomain-specific manner [3].
  • Null mutation of Dlx-2 results in abnormal morphogenesis of proximal first and second branchial arch derivatives and abnormal differentiation in the forebrain [4].
  • These results show that Dlx-2 controls development of the branchial arches and the forebrain and suggests its role in craniofacial evolution [4].

Biological context of Dlx2


Anatomical context of Dlx2

  • Notably, in animals carrying either of these genetic backgrounds, genes such as Gsh2 and Dlx2, which are expressed pan-ventrally, as well as Nkx2.1, which demarcates the ventral most aspect of the telencephalon, appear to be largely restored to their wild-type patterns of expression [9].
  • By 48 h post-electroporation, the majority of transfected cells formed multicellular aggregates that were found adjacent to the basal side of the neural tube and very few Dlx2 expressing cells migrated to the level of the branchial arches [5].
  • Ectopic expression of Dlx2 within the neural tube beginning prior to emigration of neural crest cells at E1.25 drastically inhibits the migration of transfected cells and induces aggregation of transfected neuroepithelial cells within the neural tube at 24 h post-electroporation [5].
  • Our results support a possible role for Dlx1 and Dlx2 in inner retinal development and in the terminal differentiation and/or maintenance of INL interneurons and ganglion cells in the adult [10].
  • We also examined the involvement of Dlx2 in the bone morphogenetic protein-2 (BMP-2) signaling to the type II collagen gene, Col2alpha1, in this cell line [6].

Associations of Dlx2 with chemical compounds

  • This approach showed that ectopic expression of Dlx2 and Dlx5 induced the expression of glutamic acid decarboxylases (GADs), the enzymes that synthesize GABA [11].
  • Previous reports focusing on embryogenesis of telencephalic neurons in Dlx-1 and Dlx-2 null mice suggested a specific role for these genes in expression of the OB dopamine (DA) phenotype [12].

Regulatory relationships of Dlx2


Other interactions of Dlx2

  • Dlx-1 and Dlx-2 are expressed in two separate regions of the forebrain in an identical pattern [14].
  • Dlx1 and Dlx2 are detected in retinal neuroprogenitors by embryonic day (E) 12.5 (Eisenstat et al. [1999] J. Comp. Neurol. 217-237) [10].
  • In contrast to Dlx-2 and other vertebrate Distal-less genes, Dlx-3 is not expressed in the central nervous system and is expressed in a highly restricted region of the branchial arches [15].
  • Thus, the signalling molecules BMP4 and FGF8 provide the mechanism for maintaining the strict epithelial and mesenchymal expression domains of Dlx2 in the first arch [8].
  • Investigation of pan-ventral markers such as Dlx2 also shows that, unlike Shh(-/-), a broad domain of expression of this gene is observed in Shh(-/-);Pax6(-/-) mice [16].

Analytical, diagnostic and therapeutic context of Dlx2


  1. Dlx gene expression during chick inner ear development. Brown, S.T., Wang, J., Groves, A.K. J. Comp. Neurol. (2005) [Pubmed]
  2. N-terminal fatty-acylation of sonic hedgehog enhances the induction of rodent ventral forebrain neurons. Kohtz, J.D., Lee, H.Y., Gaiano, N., Segal, J., Ng, E., Larson, T., Baker, D.P., Garber, E.A., Williams, K.P., Fishell, G. Development (2001) [Pubmed]
  3. The Evf-2 noncoding RNA is transcribed from the Dlx-5/6 ultraconserved region and functions as a Dlx-2 transcriptional coactivator. Feng, J., Bi, C., Clark, B.S., Mady, R., Shah, P., Kohtz, J.D. Genes Dev. (2006) [Pubmed]
  4. Null mutation of Dlx-2 results in abnormal morphogenesis of proximal first and second branchial arch derivatives and abnormal differentiation in the forebrain. Qiu, M., Bulfone, A., Martinez, S., Meneses, J.J., Shimamura, K., Pedersen, R.A., Rubenstein, J.L. Genes Dev. (1995) [Pubmed]
  5. Dlx2 over-expression regulates cell adhesion and mesenchymal condensation in ectomesenchyme. McKeown, S.J., Newgreen, D.F., Farlie, P.G. Dev. Biol. (2005) [Pubmed]
  6. Bone morphogenetic protein-2 (BMP-2) signaling to the Col2alpha1 gene in chondroblasts requires the homeobox gene Dlx-2. Xu, S.C., Harris, M.A., Rubenstein, J.L., Mundy, G.R., Harris, S.E. DNA Cell Biol. (2001) [Pubmed]
  7. Dlx1 and Dlx2 function is necessary for terminal differentiation and survival of late-born retinal ganglion cells in the developing mouse retina. de Melo, J., Du, G., Fonseca, M., Gillespie, L.A., Turk, W.J., Rubenstein, J.L., Eisenstat, D.D. Development (2005) [Pubmed]
  8. Independent regulation of Dlx2 expression in the epithelium and mesenchyme of the first branchial arch. Thomas, B.L., Liu, J.K., Rubenstein, J.L., Sharpe, P.T. Development (2000) [Pubmed]
  9. Dorsoventral patterning is established in the telencephalon of mutants lacking both Gli3 and Hedgehog signaling. Rallu, M., Machold, R., Gaiano, N., Corbin, J.G., McMahon, A.P., Fishell, G. Development (2002) [Pubmed]
  10. Dlx1, Dlx2, Pax6, Brn3b, and Chx10 homeobox gene expression defines the retinal ganglion and inner nuclear layers of the developing and adult mouse retina. de Melo, J., Qiu, X., Du, G., Cristante, L., Eisenstat, D.D. J. Comp. Neurol. (2003) [Pubmed]
  11. Ectopic expression of the Dlx genes induces glutamic acid decarboxylase and Dlx expression. Stühmer, T., Anderson, S.A., Ekker, M., Rubenstein, J.L. Development (2002) [Pubmed]
  12. Dlx-1 and Dlx-2 expression in the adult mouse brain: relationship to dopaminergic phenotypic regulation. Saino-Saito, S., Berlin, R., Baker, H. J. Comp. Neurol. (2003) [Pubmed]
  13. DLX-1, DLX-2, and DLX-5 expression define distinct stages of basal forebrain differentiation. Eisenstat, D.D., Liu, J.K., Mione, M., Zhong, W., Yu, G., Anderson, S.A., Ghattas, I., Puelles, L., Rubenstein, J.L. J. Comp. Neurol. (1999) [Pubmed]
  14. Spatially restricted expression of Dlx-1, Dlx-2 (Tes-1), Gbx-2, and Wnt-3 in the embryonic day 12.5 mouse forebrain defines potential transverse and longitudinal segmental boundaries. Bulfone, A., Puelles, L., Porteus, M.H., Frohman, M.A., Martin, G.R., Rubenstein, J.L. J. Neurosci. (1993) [Pubmed]
  15. Differential and overlapping expression domains of Dlx-2 and Dlx-3 suggest distinct roles for Distal-less homeobox genes in craniofacial development. Robinson, G.W., Mahon, K.A. Mech. Dev. (1994) [Pubmed]
  16. Removal of Pax6 partially rescues the loss of ventral structures in Shh null mice. Fuccillo, M., Rutlin, M., Fishell, G. Cereb. Cortex (2006) [Pubmed]
  17. Biomineralization, life-time of odontogenic cells and differential expression of the two homeobox genes MSX-1 and DLX-2 in transgenic mice. Lézot, F., Thomas, B., Hotton, D., Forest, N., Orestes-Cardoso, S., Robert, B., Sharpe, P., Berdal, A. J. Bone Miner. Res. (2000) [Pubmed]
  18. Altered forebrain and hindbrain development in mice mutant for the Gsh-2 homeobox gene. Szucsik, J.C., Witte, D.P., Li, H., Pixley, S.K., Small, K.M., Potter, S.S. Dev. Biol. (1997) [Pubmed]
  19. The spatial localization of Dlx-2 during tooth development. Thomas, B.L., Porteus, M.H., Rubenstein, J.L., Sharpe, P.T. Connect. Tissue Res. (1995) [Pubmed]
WikiGenes - Universities