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Gene Review

Vsx2  -  visual system homeobox 2

Mus musculus

Synonyms: Ceh-10 homeodomain-containing homolog, Chx10, Homeobox protein CHX10, Hox-10, Hox10, ...
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Disease relevance of Chx10

  • In agreement with this, misexpression of Chx10 alone with retrovirus in the retinal explant cultures induced generation of the inner nuclear layer cells, including Müller glia, but few of them were mature bipolar cells [1].
  • In addition, the absence of Chx10 appears to affect the development of late-born cells more than that of early-born cells, in that a low number of rods develops, whereas formation of ganglion, amacrine, and cone cells is relatively unaffected [2].

High impact information on Chx10

  • Here we show that mice carrying the OrJ allele have a premature stop codon in the homeobox of the Chx10 gene, a gene expressed at high levels in uncommitted retinal progenitor cells and mature bipolar cells [3].
  • In mice lacking Hb9, the genetic profile of MNs is significantly altered, particularly by upregulation of Chx10, a gene normally restricted to a class of ventral interneurons [4].
  • Chx10 transcripts are also detected in regions of the developing thalamus, hindbrain, and ventral spinal cord [5].
  • Similarly, the expression of Chx10 and Bmp4, genes required for retinal precursor proliferation and for lens development, respectively, was also decreased in the optic vesicles of Frs2alpha(2F/2F) mice [6].
  • Bipolar cell fate was not altered in the absence of Vsx1 function, because the pan-bipolar markers Chx10 and Ret-B1 continued to be expressed in inner nuclear layer neurons labeled by the Vsx1-targeting reporter gene, tauLacZ [7].

Biological context of Chx10

  • Previous analysis of mutant mice has revealed that the bHLH genes Mash1 and Math3, and the homeobox gene Chx10 are essential for generation of bipolar cells, the interneurons present in the inner nuclear layer of the retina [1].
  • Furthermore, our immunohistochemical analysis suggests the existence of diverse factors (e.g., Pax6 and Chx10) within the retinal cells that differentially and inappropriately activate the two transgenes [8].
  • Insufficient cell number is a primary cause of failed retinal development in the Chx10 mutant mouse [9].
  • Genetic rescue of cell number in a mouse model of microphthalmia: interactions between Chx10 and G1-phase cell cycle regulators [9].
  • Misexpression of Chx10 in the developing retinal pigmented epithelium (RPE) caused downregulation of Mitf, Tfec, and associated pigment markers, leading to a nonpigmented RPE [10].

Anatomical context of Chx10

  • It is required for retinal progenitor cell proliferation as well as formation of bipolar cells, a type of retinal interneuron. or(J) (ocular retardation) mice, which are Chx10 null mutants, are microphthalmic and show expanded and abnormal peripheral structures, including the ciliary body [10].
  • During vertebrate eye development, the cells of the optic vesicle (OV) become either neuroretinal progenitors expressing the transcription factor Chx10, or retinal pigment epithelium (RPE) progenitors expressing the transcription factor Mitf [11].
  • The reporter expression was faithful to the endogenous retinal Chx10 expression pattern and revealed a previously unappreciated locus of Chx10 expression in a subset of Müller glial cells [12].
  • Vsx-2 is initially expressed by proliferating neuroepithelial cells of the presumptive neural retina, then it is down-regulated as differentiation begins, and it is finally reexpressed at later stages of differentiation in a subset of cells, presumed to be bipolar cells, in the inner nuclear layer [13].
  • In the absence of Hox10 function, no lumbar vertebrae are formed [14].

Regulatory relationships of Chx10

  • We report here that Mitf is expressed ectopically in the Chx10(or-J/or-J) neuroretina (NR), demonstrating that Chx10 normally represses the neuroretinal expression of Mitf [11].
  • Nestin expression in the retina is restricted to intermediate/late RPC subsets, and genetic evidence is presented that demonstrates that Chx10 represses Nestin expression in early RPCs [15].
  • Correlation of overall expression patterns between different retinal cell markers and Ptn in situ hybridization suggest that Ptn transcripts are initially expressed in progenitor cells then in postmitotic precursors of the INL expressing the Chx10 gene, and later in some differentiated retinal M??ller glial (RMG) cells and rod-bipolar cells [16].

Other interactions of Chx10

  • We conclude that Mab21l2 expression is essential for optic vesicle growth and formation of the optic cup, its absence causing reduced expression of Chx10 [17].
  • These data link Chx10 and Mitf to maintenance of the neural retina and RPE fates respectively [10].
  • Whereas Chx10 is expressed both in retinal progenitors in the developing eye and apparently in all bipolar cells of the mature retina, Vsx1 expression is first detected in the eye at postnatal day 5, where it is restricted to cone bipolar cells [18].
  • Analysis of the Brn-2 expression pattern shows that it is expressed in intermediate and late RPCs, but not early RPCs, and thus partially overlaps with expression of the reporter activated by the defined Chx10 enhancer [15].
  • These data identify Brn-2 as a new marker of subsets of RPCs and suggest a mechanism by which a combination of POU factors and Chx10 define RPC gene expression patterns, such as that of Nestin [15].


  1. Roles of homeobox and bHLH genes in specification of a retinal cell type. Hatakeyama, J., Tomita, K., Inoue, T., Kageyama, R. Development (2001) [Pubmed]
  2. Delayed expression of the Crx gene and photoreceptor development in the Chx10-deficient retina. Rutherford, A.D., Dhomen, N., Smith, H.K., Sowden, J.C. Invest. Ophthalmol. Vis. Sci. (2004) [Pubmed]
  3. Ocular retardation mouse caused by Chx10 homeobox null allele: impaired retinal progenitor proliferation and bipolar cell differentiation. Burmeister, M., Novak, J., Liang, M.Y., Basu, S., Ploder, L., Hawes, N.L., Vidgen, D., Hoover, F., Goldman, D., Kalnins, V.I., Roderick, T.H., Taylor, B.A., Hankin, M.H., McInnes, R.R. Nat. Genet. (1996) [Pubmed]
  4. Active suppression of interneuron programs within developing motor neurons revealed by analysis of homeodomain factor HB9. Thaler, J., Harrison, K., Sharma, K., Lettieri, K., Kehrl, J., Pfaff, S.L. Neuron (1999) [Pubmed]
  5. Developmental expression of a novel murine homeobox gene (Chx10): evidence for roles in determination of the neuroretina and inner nuclear layer. Liu, I.S., Chen, J.D., Ploder, L., Vidgen, D., van der Kooy, D., Kalnins, V.I., McInnes, R.R. Neuron (1994) [Pubmed]
  6. Tyrosine phosphorylation sites on FRS2alpha responsible for Shp2 recruitment are critical for induction of lens and retina. Gotoh, N., Ito, M., Yamamoto, S., Yoshino, I., Song, N., Wang, Y., Lax, I., Schlessinger, J., Shibuya, M., Lang, R.A. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  7. Control of late off-center cone bipolar cell differentiation and visual signaling by the homeobox gene Vsx1. Chow, R.L., Volgyi, B., Szilard, R.K., Ng, D., McKerlie, C., Bloomfield, S.A., Birch, D.G., McInnes, R.R. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  8. MyoD-lacZ transgenes are early markers in the neural retina, but MyoD function appears to be inhibited in the developing retinal cells. Kablar, B. Int. J. Dev. Neurosci. (2004) [Pubmed]
  9. Genetic rescue of cell number in a mouse model of microphthalmia: interactions between Chx10 and G1-phase cell cycle regulators. Green, E.S., Stubbs, J.L., Levine, E.M. Development (2003) [Pubmed]
  10. Transdifferentiation of the retina into pigmented cells in ocular retardation mice defines a new function of the homeodomain gene Chx10. Rowan, S., Chen, C.M., Young, T.L., Fisher, D.E., Cepko, C.L. Development (2004) [Pubmed]
  11. Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Horsford, D.J., Nguyen, M.T., Sellar, G.C., Kothary, R., Arnheiter, H., McInnes, R.R. Development (2005) [Pubmed]
  12. Genetic analysis of the homeodomain transcription factor Chx10 in the retina using a novel multifunctional BAC transgenic mouse reporter. Rowan, S., Cepko, C.L. Dev. Biol. (2004) [Pubmed]
  13. Vsx-1 and Vsx-2: differential expression of two paired-like homeobox genes during zebrafish and goldfish retinogenesis. Passini, M.A., Levine, E.M., Canger, A.K., Raymond, P.A., Schechter, N. J. Comp. Neurol. (1997) [Pubmed]
  14. Hox10 and Hox11 genes are required to globally pattern the mammalian skeleton. Wellik, D.M., Capecchi, M.R. Science (2003) [Pubmed]
  15. A POU factor binding site upstream of the Chx10 homeobox gene is required for Chx10 expression in subsets of retinal progenitor cells and bipolar cells. Rowan, S., Cepko, C.L. Dev. Biol. (2005) [Pubmed]
  16. Involvement of Pleiotrophin in CNTF-mediated differentiation of the late retinal progenitor cells. Roger, J., Brajeul, V., Thomasseau, S., Hienola, A., Sahel, J.A., Guillonneau, X., Goureau, O. Dev. Biol. (2006) [Pubmed]
  17. Requirement for Mab21l2 during development of murine retina and ventral body wall. Yamada, R., Mizutani-Koseki, Y., Koseki, H., Takahashi, N. Dev. Biol. (2004) [Pubmed]
  18. Vsx1, a rapidly evolving paired-like homeobox gene expressed in cone bipolar cells. Chow, R.L., Snow, B., Novak, J., Looser, J., Freund, C., Vidgen, D., Ploder, L., McInnes, R.R. Mech. Dev. (2001) [Pubmed]
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