Transcriptional Regulation of Their Survival:

The Engrailed Homeobox Genes
  • Horst H. SimonEmail author
  • Kambiz N. Alavian
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 651)


The mammalian Engrailed genes1, 2, 3 were originally cloned by their sequence homology to the Drosophila engrailed. Orthologous genes of these homeobox transcription factors are found throughout the animal kingdom including all investigated vertebrate species.4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17 They take part in regionalization of the early embryo and later participate in neuronal specification.18, 19, 20, 21 Engrailed was first mentioned as a spontaneously occurring mutation in Drosophila melanogaster 1920′,22 disrupting the normal development of thoracic segments, leading to scutellar modifications, an addition sex comb and malformation of the wings.23,24 Differences in protein length between orthologues and paralogues are significant reaching from 552 amino acids in drosophila to 261 for the zebrafish engrailed-2. Despite these large differences on the sequence level the genes are functional conserved even over different phyla. When the mouse En1 coding sequence is replaced by its paralogue En2 or by its drosophila homologue, the otherwise lethal phenotype of the En1 null mutation with large midbrain deficiencies is rescued resulting in a viable mouse.25,26 These experiments suggest a irreplaceable role for the engrailed genes in bilateria.


Expression Domain Midbrain Dopaminergic Neuron Homeobox Transcription Factor Mesencephalic Dopaminergic Neuron Anterior Neural Plate 
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Copyright information

© Landes Bioscience and Springer Science+Business Media 2009

Authors and Affiliations

  1. 1.Department of Neuroanatomy, Interdisciplinary Center for Neuroscience (IZN)University of HeidelbergHeidelberg

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