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@ARTICLE{Zhang:138219,
author = {Zhang, Jingzhong and Götz, Sebastian and Vogt-Weisenhorn,
Daniela and Simeone, Antonio and Wurst, Wolfgang and
Prakash, Nilima},
title = {{A} {WNT}1-regulated developmental gene cascade prevents
dopaminergic neurodegeneration in adult {E}n1(+/-) mice.},
journal = {Neurobiology of disease},
volume = {82},
issn = {0969-9961},
address = {Orlando, Fla.},
publisher = {Academic Press},
reportid = {DZNE-2020-04541},
pages = {32-45},
year = {2015},
abstract = {The protracted and age-dependent degeneration of dopamine
(DA)-producing neurons of the Substantia nigra pars compacta
(SNc) and ventral tegmental area (VTA) in the mammalian
midbrain is a hallmark of human Parkinson's Disease (PD) and
of certain genetic mouse models of PD, such as mice
heterozygous for the homeodomain transcription factor
Engrailed 1 (En1(+/-) mice). Neurotoxin-based animal models
of PD, in contrast, are characterized by the fast and partly
reversible degeneration of the SNc and VTA DA neurons. The
secreted protein WNT1 was previously shown to be strongly
induced in the neurotoxin-injured adult ventral midbrain
(VM), and to protect the SNc and VTA DA neurons from cell
death in this context. We demonstrate here that the
sustained and ectopic expression of Wnt1 in the SNc and VTA
DA neurons of En1(+/Wnt1) mice also protected these
genetically affected En1 heterozygote (En1(+/-)) neurons
from their premature degeneration in the adult mouse VM. We
identified a developmental gene cascade that is up-regulated
in the adult En1(+/Wnt1) VM, including the direct
WNT1/β-catenin signaling targets Lef1, Lmx1a, Fgf20 and
Dkk3, as well as the indirect targets Pitx3 (activated by
LMX1A) and Bdnf (activated by PITX3). We also show that the
secreted neurotrophin BDNF and the secreted WNT modulator
DKK3, but not the secreted growth factor FGF20, increased
the survival of En1 mutant dopaminergic neurons in vitro.
The WNT1-mediated signaling pathway and its downstream
targets BDNF and DKK3 might thus provide a useful means to
treat certain genetic and environmental (neurotoxic) forms
of human PD.},
keywords = {Adaptor Proteins, Signal Transducing / Animals /
Brain-Derived Neurotrophic Factor: genetics / Brain-Derived
Neurotrophic Factor: metabolism / Cell Differentiation:
genetics / Dopaminergic Neurons: metabolism / Dopaminergic
Neurons: pathology / Gene Expression Regulation,
Developmental / Homeodomain Proteins: genetics /
Intercellular Signaling Peptides and Proteins: genetics /
Intercellular Signaling Peptides and Proteins: metabolism /
Mice / Mice, Transgenic / Nerve Degeneration: genetics /
Nerve Degeneration: metabolism / Nerve Degeneration:
pathology / Parkinson Disease: metabolism / Parkinson
Disease: pathology / Signal Transduction: genetics /
Substantia Nigra: metabolism / Substantia Nigra: pathology /
Up-Regulation / Ventral Tegmental Area: metabolism / Ventral
Tegmental Area: pathology / Wnt1 Protein: metabolism /
Brain-Derived Neurotrophic Factor (NLM Chemicals) / Dkk3
protein, mouse (NLM Chemicals) / En1 protein, mouse (NLM
Chemicals) / Homeodomain Proteins (NLM Chemicals) /
Intercellular Signaling Peptides and Proteins (NLM
Chemicals) / Wnt1 Protein (NLM Chemicals)},
cin = {Ext HZM / AG Wurst},
ddc = {570},
cid = {I:(DE-2719)5000050 / I:(DE-2719)1140001},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:26049140},
doi = {10.1016/j.nbd.2015.05.015},
url = {https://pub.dzne.de/record/138219},
}