Proximity proteomics reveals unique and shared pathological features between multiple system atrophy and Parkinson’s disease.

Publication date: Mar 23, 2025

Synucleinopathies such as Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are neurodegenerative diseases with shared clinical and pathological features. Aggregates of alpha-synuclein (αsyn) phosphorylated at serine 129 (PSER129) are hallmarks of synucleinopathies, which, for PD/DLB, are found predominantly in neurons, whereas in MSA, aggregates are primarily found in oligodendroglia. It remains unclear whether the distinct pathological presentations of PD/DLB and MSA are manifestations of unique or shared pathological processes. Using the in-situ proximity labeling technique of biotinylation by antibody recognition (BAR), we compared aggregated αsyn-interactomes (BAR-PSER129) and total αsyn-interactomes (BAR-MJFR1) between MSA (n = 5) and PD/DLB (n = 10) in forebrain and midbrain structures. Comparison between MSA and PD/DLB-enriched proteins revealed 79 PD/DLB-differentially abundant proteins and only three MSA-differentially abundant proteins (CBR1, CRYAB, and GFAP). Pathway enrichment analysis revealed that vesicle/SNARE-associated pathways dominated PD/DLB interactions, whereas MSA was strongly enriched for metabolic/catabolic, iron, and cellular oxidant detoxification pathways. A subnetwork of cytosolic antioxidant enzymes called peroxiredoxins drove cellular detoxification pathway enrichment in MSA. A network of 26 proteins, including neuronal-specific proteins (e. g., SYNGR3) with HSPA8 at the core, was shared between MSA and DLB/PD. Extracellular exosome pathways were universally enriched regardless of the disease or BAR target protein. In conclusion, synucleinopathies have divergent and convergent αsyn-aggregate interactions, indicating unique and shared pathogenic mechanisms. MSA uniquely involves oxidant detoxification processes in glial cells, while vesicular processes in neurons dominate PD/DLB. Shared interactions, specifically SYNGR3, between MSA and PD/DLB suggest that neuronal axons are the origin of both diseases. In conclusion, we provide αsyn protein interaction maps for two distinct synucleinopathies.

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Concepts Keywords
Cryab Aged
Neurodegenerative Aged, 80 and over
Parkinson alpha-Synuclein
Proteins alpha-Synuclein
Pser129 Antioxidant
Female
Humans
Lewy Body Disease
Male
Middle Aged
Multiple System Atrophy
Neurodegeneration
Parkinson Disease
Proteomics
Proximity proteomics
SNARE complex
Spatial omics

Semantics

Type Source Name
disease MESH multiple system atrophy
disease MESH Parkinson’s disease
disease MESH Synucleinopathies
disease MESH dementia
disease MESH neurodegenerative diseases
pathway REACTOME Neurodegenerative Diseases
drug DRUGBANK Serine
disease MESH pathological processes
drug DRUGBANK Iron
pathway REACTOME Reproduction
disease MESH atrophy
disease MESH Movement Disorders
drug DRUGBANK Formaldehyde
drug DRUGBANK Phosphate ion
drug DRUGBANK Dimethyl sulfoxide
drug DRUGBANK Glycerin
disease MESH death
disease MESH Corticobasal degeneration
disease MESH parkinsonism
disease MESH tauopathy
drug DRUGBANK Sodium Citrate
drug DRUGBANK Hydrogen peroxide
drug DRUGBANK Tretamine
drug DRUGBANK Abacavir
drug DRUGBANK Sodium acetate
drug DRUGBANK Imidazole
drug DRUGBANK Tromethamine
drug DRUGBANK Esomeprazole
drug DRUGBANK Sodium borate
drug DRUGBANK Sodium lauryl sulfate
drug DRUGBANK Edetic Acid
drug DRUGBANK Ademetionine
drug DRUGBANK Ethanol
drug DRUGBANK Acetic acid
drug DRUGBANK Water
drug DRUGBANK Trypsin
drug DRUGBANK Coenzyme M
drug DRUGBANK Flunarizine
drug DRUGBANK Methylergometrine
drug DRUGBANK Isoxaflutole
drug DRUGBANK Hyaluronic acid
drug DRUGBANK Biotin
drug DRUGBANK Trestolone
drug DRUGBANK Proline
drug DRUGBANK Pidolic Acid
disease MESH shock
drug DRUGBANK Hexocyclium
disease MESH oxidative stress
pathway KEGG Oxidative phosphorylation
drug DRUGBANK Glutamic Acid
drug DRUGBANK ATP
disease MESH dissociation
drug DRUGBANK ANX-510
drug DRUGBANK Potassium
disease MESH pathogenesis
drug DRUGBANK Ubiquinol
disease MESH amyotrophic lateral sclerosis
pathway KEGG Amyotrophic lateral sclerosis
pathway KEGG Synaptic vesicle cycle
pathway REACTOME Protein localization
drug DRUGBANK Dopamine
pathway KEGG Endocytosis
pathway REACTOME Macroautophagy
pathway REACTOME Autophagy
drug DRUGBANK Ubidecarenone
drug DRUGBANK Indoleacetic acid
drug DRUGBANK Gold
drug DRUGBANK Calcium
disease MESH motor neuron disease
disease MESH frontotemporal dementia
disease MESH Lewy body disease
disease MESH ischemia
pathway KEGG Proteasome
disease MESH striatonigral degeneration
disease MESH olivopontocerebellar atrophy
disease MESH shy drager syndrome
drug DRUGBANK Rotenone
pathway REACTOME Signal amplification
disease MESH progressive supranuclear palsy
drug DRUGBANK Glutathione
drug DRUGBANK Thiocolchicoside
pathway KEGG Parkinson disease
drug DRUGBANK Ilex paraguariensis leaf

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