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@ARTICLE{Redl:281869,
author = {Redl, Stefan and Zweydorf, Felix and Gloeckner, Christian J
and Posadas, Inmaculada and Ceña, Valentín and Hess,
Michael W and Piccoli, Giovanni and Pérez-Carrión, María
Dolores},
title = {{T}he {E}193{K} {LRRK}2 mutation interferes with the
autophagosome processing through the impairment of the
{LRRK}2-{D}ynein-1 complex.},
journal = {Scientific reports},
volume = {15},
number = {1},
issn = {2045-2322},
address = {[London]},
publisher = {Springer Nature},
reportid = {DZNE-2025-01240},
pages = {39117},
year = {2025},
abstract = {Parkinson's disease (PD) is a neurodegenerative pathology
characterized by movement-associated symptoms due to the
selective loss of dopaminergic neurons in the substantia
nigra pars compacta. Autophagy is an essential mechanism
that restores homeostasis and promotes cell survival.
Mutations in the Leucine-Rich Repeat Kinase 2 (LRRK2) gene
are among the most common in the familial cases. The LRRK2
E193K mutation falls in the Armadillo (ARM) domain and
modifies LRRK2 interactome. The role of LRRK2 in autophagy
has been widely explored, but the impact of E193K mutation
on autophagy remains unknown. We found that the E193K
variant increases autophagy in primary fibroblasts obtained
from an E193K carrier. By cryo-based electron microscopy we
observed that E193K fibroblasts present a higher amount of
phagophores/autophagosomes. We showed that LRRK2 binds to
the Dynein-1 complex, an essential regulator of retrograde
transport of autophagosomes. Noteworthy, the E193K mutation
jeopardizes this interaction and increases the cellular
sensitivity to 1-methyl-4-phenylpyridinium (MPP+) toxin in
fibroblasts as well as in a heterologous cell model. Our
study reveals that the LRRK2 E193K variant influences the
autophagic regulation and suggests that the dysregulation of
the LRRK2-Dynein-1 complex causes autophagic defects and,
eventually, cell death.},
keywords = {Leucine-Rich Repeat Serine-Threonine Protein Kinase-2:
genetics / Leucine-Rich Repeat Serine-Threonine Protein
Kinase-2: metabolism / Humans / Autophagosomes: metabolism /
Fibroblasts: metabolism / Autophagy: genetics / Mutation /
Parkinson Disease: genetics / Parkinson Disease: metabolism
/ Parkinson Disease: pathology / Dyneins: metabolism /
Dyneins: genetics / Protein Binding / Autophagy (Other) /
Dynein-1 complex (Other) / LRRK2 protein (Other) / Organelle
morphometry (Other) / Parkinson´s disease (Other) /
Ultrastructure (Other) / Leucine-Rich Repeat
Serine-Threonine Protein Kinase-2 (NLM Chemicals) / LRRK2
protein, human (NLM Chemicals) / Dyneins (NLM Chemicals)},
cin = {AG Gloeckner},
ddc = {600},
cid = {I:(DE-2719)1210007},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41203815},
pmc = {pmc:PMC12594833},
doi = {10.1038/s41598-025-26716-4},
url = {https://pub.dzne.de/record/281869},
}