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@ARTICLE{LaVitola:273935,
author = {La Vitola, Pietro and Szegoe, Eva and Pinto-Costa, Rita and
Rollar, Angela and Harbachova, Eugenia and Schapira, Anthony
Hv and Ulusoy, Ayse and Di Monte, Donato A},
title = {{M}itochondrial oxidant stress promotes α-synuclein
aggregation and spreading in mice with mutated
glucocerebrosidase.},
journal = {npj Parkinson's Disease},
volume = {10},
number = {1},
issn = {2373-8057},
address = {London [u.a.]},
publisher = {Nature Publ. Group},
reportid = {DZNE-2024-01409},
pages = {233},
year = {2024},
abstract = {In this study, heterozygous expression of a common
Parkinson-associated GBA1 variant, the L444P mutation, was
found to exacerbate α-synuclein aggregation and spreading
in a mouse model of Parkinson-like pathology targeting
neurons of the medullary vagal system. These neurons were
also shown to become more vulnerable to oxidative and
nitrative stress after L444P expression. The latter
paralleled neuronal formation of reactive oxygen species and
led to a pronounced accumulation of nitrated α-synuclein. A
causal relationship linked mutation-induced
oxidative/nitrative stress to enhanced α-synuclein
aggregation and spreading that could indeed be rescued by
neuronal overexpression of mitochondrial superoxide
dismutase 2. Further evidence supported a key involvement of
mitochondria as sources of reactive oxygen species as well
as targets of oxidative and nitrative damage within
L444P-expressing neurons. These findings support the
conclusion that enhanced vulnerability to mitochondrial
oxidative stress should be considered an important mechanism
predisposing to pathology conversion in carriers of GBA1
mutations.},
cin = {AG Di Monte},
ddc = {610},
cid = {I:(DE-2719)1013008},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39663354},
pmc = {pmc:PMC11634889},
doi = {10.1038/s41531-024-00842-8},
url = {https://pub.dzne.de/record/273935},
}