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@ARTICLE{Knab:274040,
author = {Knab, Felix and Guaitoli, Giambattista and Jarboui, Mohamed
Ali and von Zweydorf, Felix and Isik, Fatma Busra and Klose,
Franziska and Rajkumar, Anto Praveen and Gasser, Thomas and
Gloeckner, Christian Johannes},
title = {{T}he cellular and extracellular proteomic signature of
human dopaminergic neurons carrying the {LRRK}2 {G}2019{S}
mutation.},
journal = {Frontiers in neuroscience},
volume = {18},
issn = {1662-4548},
address = {Lausanne},
publisher = {Frontiers Research Foundation},
reportid = {DZNE-2025-00021},
pages = {1502246},
year = {2024},
abstract = {Extracellular vesicles are easily accessible in various
biofluids and allow the assessment of disease-related
changes in the proteome. This has made them a promising
target for biomarker studies, especially in the field of
neurodegeneration where access to diseased tissue is very
limited. Genetic variants in the LRRK2 gene have been linked
to both familial and sporadic forms of Parkinson's disease.
With LRRK2 inhibitors entering clinical trials, there is an
unmet need for biomarkers that reflect LRRK2-specific
pathology and target engagement.In this study, we used
induced pluripotent stem cells derived from a patient with
Parkinson's disease carrying the LRRK2 G2019S mutation and
an isogenic gene-corrected control to generate human
dopaminergic neurons. We isolated extracellular vesicles and
neuronal cell lysates and characterized their proteomic
signature using data-independent acquisition proteomics.
Then, we performed differential expression analysis to
identify dysregulated proteins in the mutated line. We used
Metascape and gene ontology enrichment analysis on the
dysregulated proteomes to identify changes in associated
functional networks.We identified 595 significantly
differentially regulated proteins in extracellular vesicles
and 3,205 in cell lysates. We visualized functionally
relevant protein-protein interaction networks and identified
key regulators within the dysregulated proteomes. Using gene
ontology, we found a close association with biological
processes relevant to neurodegeneration and Parkinson's
disease. Finally, we focused on proteins that were
dysregulated in both the extracellular and cellular
proteomes. We provide a list of ten biomarker candidates
that are functionally relevant to neurodegeneration and
linked to LRRK2-associated pathology, for example, the sonic
hedgehog signaling molecule, a protein that has tightly been
linked to LRRK2-related disruption of cilia function.In
conclusion, we characterized the cellular and extracellular
proteome of dopaminergic neurons carrying the LRRK2 G2019S
mutation and proposed an experimentally based list of
biomarker candidates for future studies.},
keywords = {LRRK2 (Other) / Parkinson’s disease (Other) / biomarker
(Other) / extracellular vesicle (Other) / induced
pluripotent stem cell (Other)},
cin = {AG Gloeckner / AG Gasser},
ddc = {610},
cid = {I:(DE-2719)1210007 / I:(DE-2719)1210000},
pnm = {352 - Disease Mechanisms (POF4-352) / 353 - Clinical and
Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-352 / G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39726830},
pmc = {pmc:PMC11669673},
doi = {10.3389/fnins.2024.1502246},
url = {https://pub.dzne.de/record/274040},
}