Progressive loss of dopaminergic neurons in the ventral midbrain of adult mice heterozygote for Engrailed1: a new genetic model for Parkinson's disease?

Parkinsonism Relat Disord. 2008;14 Suppl 2:S107-11. doi: 10.1016/j.parkreldis.2008.04.007. Epub 2008 Jun 27.


Engrailed1 is a developmental gene of the homeogene family that controls the survival of midbrain dopaminergic neurons throughout life. Since these neurons have been crucially implicated in Parkinson's disease (PD), transgenic mice lacking one En1 allele could be of particular interest for the development of an animal model for PD. We showed in En1+/- mice, some traits reminiscent of PD such as (1) a progressive loss of mesencephalic dopaminergic (DA) neurons, and (2) motor deficits, anhedonia, decreased social interactions and depression-like behaviours. Further validation is needed, but these first results suggest that En1+/- mice could provide a promising model for the study of PD.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Disease Models, Animal*
  • Dopamine / metabolism*
  • Gene Expression Regulation, Developmental / genetics
  • Homeodomain Proteins / genetics*
  • Humans
  • Mesencephalon / pathology*
  • Mice
  • Mice, Knockout
  • Neurons / metabolism*
  • Neurons / pathology
  • Parkinson Disease* / genetics
  • Parkinson Disease* / metabolism
  • Parkinson Disease* / pathology
  • Tyrosine 3-Monooxygenase / metabolism


  • En1 protein, mouse
  • Homeodomain Proteins
  • Tyrosine 3-Monooxygenase
  • Dopamine