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Gene Review

SIX2  -  SIX homeobox 2

Homo sapiens

Synonyms: Homeobox protein SIX2, Sine oculis homeobox homolog 2
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High impact information on SIX2

  • In this paper, we report that functional inactivation of the homeobox gene Six2 results in premature and ectopic differentiation of mesenchymal cells into epithelia and depletion of the progenitor cell population within the metanephric mesenchyme [1].
  • The expression pattern of SIX2 and its localisation to chromosome 2p15-p16 will be of use in assessing its candidacy in human developmental disorders [2].
  • SIX2 is widely expressed in the late first-trimester fetus, but has a limited range of expression sites in the adult [2].
  • The recently described murine homeobox genes, Six1 and Six2, which are expressed during development in limb tendons, have also been shown to be expressed in skeletal and smooth muscle, respectively [3].

Analytical, diagnostic and therapeutic context of SIX2

  • Gain of Six2 function in cortical metanephric mesenchymal cells was sufficient to prevent their epithelial differentiation in an organ culture assay [1].


  1. Six2 is required for suppression of nephrogenesis and progenitor renewal in the developing kidney. Self, M., Lagutin, O.V., Bowling, B., Hendrix, J., Cai, Y., Dressler, G.R., Oliver, G. EMBO J. (2006) [Pubmed]
  2. Structure, mapping and expression of the human gene encoding the homeodomain protein, SIX2. Boucher, C.A., Winchester, C.L., Hamilton, G.M., Winter, A.D., Johnson, K.J., Bailey, M.E. Gene (2000) [Pubmed]
  3. Cloning of the human SIX1 gene and its assignment to chromosome 14. Boucher, C.A., Carey, N., Edwards, Y.H., Siciliano, M.J., Johnson, K.J. Genomics (1996) [Pubmed]
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