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Gene Review

EN1  -  engrailed homeobox 1

Homo sapiens

Synonyms: Homeobox protein en-1, Homeobox protein engrailed-1, Hu-En-1
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High impact information on EN1

  • Dorsoventral (DV) patterning of the vertebrate limb requires the function of the transcription factor Engrailed 1 (EN1) in the ventral ectoderm [1].
  • Cell lineage analysis of the expression of an engrailed homolog in leech embryos [2].
  • An HaeIII RFLP for the human homeo box-containing gene EN1 [3].
  • The RNA signal for both EN1 and EN2 was strongest in the cerebellar granule cell layers, white matter of the vermis and flocculus, inferior olive, arcuate nucleus, caudal raphe nuclei, corpus pontobulbare and nucleus ambiguus [4].
  • Deletions of 2q14 that include the homeobox engrailed 1 (EN1) transcription factor are compatible with a normal phenotype [5].

Biological context of EN1


Anatomical context of EN1

  • Here, we have refined the localization of EN1 to human chromosome 2q13-q21 using a mapping panel of rodent/human cell hybrids containing different regions of chromosome 2 and a lymphoblastoid cell line with an interstitial deletion, del(2)(q21-q23.2) [7].

Associations of EN1 with chemical compounds

  • Inhibition of co-translational transfer of N-linked oligosaccharides by tunicamycin produced EN1 and EN3 as intracellular species, and EN3 was sulfated and secreted [9].
  • The isocarbostyril nucleoside compound 1-(2-deoxy-beta-D-ribofuranosyl)-isocarbostyril (EN1) was a poor substrate with all the enzymes [10].

Other interactions of EN1


  1. BMP controls proximodistal outgrowth, via induction of the apical ectodermal ridge, and dorsoventral patterning in the vertebrate limb. Pizette, S., Abate-Shen, C., Niswander, L. Development (2001) [Pubmed]
  2. Cell lineage analysis of the expression of an engrailed homolog in leech embryos. Lans, D., Wedeen, C.J., Weisblat, D.A. Development (1993) [Pubmed]
  3. An HaeIII RFLP for the human homeo box-containing gene EN1. Logan, C., Joyner, A.L. Nucleic Acids Res. (1989) [Pubmed]
  4. Expression of the homeobox-containing genes EN1 and EN2 in human fetal midgestational medulla and cerebellum. Zec, N., Rowitch, D.H., Bitgood, M.J., Kinney, H.C. J. Neuropathol. Exp. Neurol. (1997) [Pubmed]
  5. Deletions of 2q14 that include the homeobox engrailed 1 (EN1) transcription factor are compatible with a normal phenotype. Barber, J.C., Maloney, V.K., Bewes, B., Wakeling, E. Eur. J. Hum. Genet. (2006) [Pubmed]
  6. Chromosomal localization of the human homeo box-containing genes, EN1 and EN2. Logan, C., Willard, H.F., Rommens, J.M., Joyner, A.L. Genomics (1989) [Pubmed]
  7. Regional assignment of the human homeobox-containing gene EN1 to chromosome 2q13-q21. Köhler, A., Logan, C., Joyner, A.L., Muenke, M. Genomics (1993) [Pubmed]
  8. Refinement of bovine chromosome 2 linkage map near the mh locus reveals rearrangements between the bovine and human genomes. Sonstegard, T.S., Kappes, S.M., Keele, J.W., Smith, T.P. Anim. Genet. (1998) [Pubmed]
  9. Tyrosine sulfation is not the last modification of entactin before its secretion from 3T3-L1 adipocytes. Aratani, Y., Kitagawa, Y. FEBS Lett. (1988) [Pubmed]
  10. Phosphorylation of isocarbostyril- and difluorophenyl-nucleoside thymidine mimics by the human deoxynucleoside kinases. Al-Madhoun, A.S., Eriksson, S., Wang, Z.X., Naimi, E., Knaus, E.E., Wiebe, L.I. Nucleosides Nucleotides Nucleic Acids (2004) [Pubmed]
  11. Joubert syndrome co-existing with partial Xp trisomy: review of the literature. Güven, G.S., Fenerci, E.Y., Deviren, A., Ozkiliç, A., Yüksel, A., Hacihanefioğlu, S. Genetic counseling (Geneva, Switzerland) (2004) [Pubmed]
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