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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Hopx  -  HOP homeobox

Mus musculus

Synonyms: 1110018K11Rik, 1200015P04Rik, 2300002F06Rik, AI848177, AW490897, ...
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Disease relevance of Hod


High impact information on Hod

  • Genetic and biochemical data indicate that Hop functions directly downstream of Nkx2-5 [4].
  • Inactivation of Hop in mice by homologous recombination results in a partially penetrant embryonic lethal phenotype with severe developmental cardiac defects involving the myocardium [4].
  • Thus Hop is regulated in the forebrain by a so far unidentified paracrine signaling factor from the roof plate [5].
  • The smallest known homeodomain protein, Homeodomain only protein (Hop), was identified and described here as a temporally and spatially restricted gene in the neurogenic regions of the developing murine CNS including the cerebral cortex [5].
  • We further demonstrate that the Hop enhancer activity is inducible ectopically in a transgenic tissue by wild-type roof plate transplantation in vitro [5].

Biological context of Hod

  • Homeobox protein Hop functions in the adult cardiac conduction system [6].
  • Recombinant viruses were constructed with these changes as single amino acid substitutions in a strain Toto 1101 background [2].
  • RESULTS: Subtractive hybridization identified transcripts encoding Hop, a short, atypical homeodomain-containing protein that had previously been shown to be an important regulator of gene expression in the heart and lung [7].

Anatomical context of Hod

  • Although lens abnormalities have yet to be identified in mice lacking Hop, the genomic sequences that regulate Hop expression in the lens may be useful for expressing exogenous transcripts selectively in fiber cells just before they fuse with their neighbors and degrade their organelles [7].

Associations of Hod with chemical compounds

  • All exhibited decreased binding to heparan sulfate and had larger plaques than Toto 1101 [2].


  1. Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeodomain protein Hop. Kook, H., Lepore, J.J., Gitler, A.D., Lu, M.M., Wing-Man Yung, W., Mackay, J., Zhou, R., Ferrari, V., Gruber, P., Epstein, J.A. J. Clin. Invest. (2003) [Pubmed]
  2. Large-plaque mutants of Sindbis virus show reduced binding to heparan sulfate, heightened viremia, and slower clearance from the circulation. Byrnes, A.P., Griffin, D.E. J. Virol. (2000) [Pubmed]
  3. Infection of neonatal mice with sindbis virus results in a systemic inflammatory response syndrome. Klimstra, W.B., Ryman, K.D., Bernard, K.A., Nguyen, K.B., Biron, C.A., Johnston, R.E. J. Virol. (1999) [Pubmed]
  4. Hop is an unusual homeobox gene that modulates cardiac development. Chen, F., Kook, H., Milewski, R., Gitler, A.D., Lu, M.M., Li, J., Nazarian, R., Schnepp, R., Jen, K., Biben, C., Runke, G., Mackay, J.P., Novotny, J., Schwartz, R.J., Harvey, R.P., Mullins, M.C., Epstein, J.A. Cell (2002) [Pubmed]
  5. A roof plate-dependent enhancer controls the expression of Homeodomain only protein in the developing cerebral cortex. Mühlfriedel, S., Kirsch, F., Gruss, P., Stoykova, A., Chowdhury, K. Dev. Biol. (2005) [Pubmed]
  6. Homeobox protein Hop functions in the adult cardiac conduction system. Ismat, F.A., Zhang, M., Kook, H., Huang, B., Zhou, R., Ferrari, V.A., Epstein, J.A., Patel, V.V. Circ. Res. (2005) [Pubmed]
  7. Identification and expression of Hop, an atypical homeobox gene expressed late in lens fiber cell terminal differentiation. Vasiliev, O., Rhodes, S.J., Beebe, D.C. Mol. Vis. (2007) [Pubmed]
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