Characterization of the Engrailed mutant mice as experimental models for Parkinson's disease.
Engrailed genes are homeodomain-containing transcription factors necessary for the development and maintenance of mesencephalic dopaminergic neurons. Deletion in the Engrailed genes has been shown to affect the survival of mesencephalic dopaminergic neurons both during development and in the adult. Here we describe for the first time a significant reduction in striatal dopamine levels in En1+/-;En2+/- mice compared with their En2+/- littermates, accompanied by a modest reduction in the number of nigral DA neurons. Our results strengthen previous evidence indicating Engrailed genes as survival factors for mature dopaminergic neurons. Furthermore, our data suggest a role for these transcription factors in the maintenance of synaptic dopaminergic neurotransmission in adult neurons.
Sgadò
Paola
P
Department of Neuroscience, University of Pisa School of Medicine, Via Roma 55, Pisa, Italy. p.sgado@meyer.it
Viaggi
Cristina
C
Fantacci
Chiara
C
Corsini
Giovanni Umberto
GU
D000818:Animals; D003342:Corpus Striatum / Q000378:metabolism; D004195:Disease Models, Animal; D018398:Homeodomain Proteins / Q000235:genetics*; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation / Q000235:genetics*; D009419:Nerve Tissue Proteins / Q000172:deficiency / Q000235:genetics*; D010300:Parkinson Disease / Q000235:genetics* / Q000473:pathology; D014446:Tyrosine 3-Monooxygenase / Q000378:metabolism; D000099266:Engrailed 2 Protein
D018398:Homeodomain Proteins; D009419:Nerve Tissue Proteins; D014446:Tyrosine 3-Monooxygenase; D000099266:Engrailed 2 Protein; C080330:En1 protein, mouse