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α-synuclein oligomers interact with ATP synthase and open the permeability transition pore in Parkinson's disease.


Type

Article

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Authors

Ludtmann, Marthe HR 
Angelova, Plamena R 
Horrocks, Mathew H 
Choi, Minee L 
Rodrigues, Margarida 

Abstract

Protein aggregation causes α-synuclein to switch from its physiological role to a pathological toxic gain of function. Under physiological conditions, monomeric α-synuclein improves ATP synthase efficiency. Here, we report that aggregation of monomers generates beta sheet-rich oligomers that localise to the mitochondria in close proximity to several mitochondrial proteins including ATP synthase. Oligomeric α-synuclein impairs complex I-dependent respiration. Oligomers induce selective oxidation of the ATP synthase beta subunit and mitochondrial lipid peroxidation. These oxidation events increase the probability of permeability transition pore (PTP) opening, triggering mitochondrial swelling, and ultimately cell death. Notably, inhibition of oligomer-induced oxidation prevents the pathological induction of PTP. Inducible pluripotent stem cells (iPSC)-derived neurons bearing SNCA triplication, generate α-synuclein aggregates that interact with the ATP synthase and induce PTP opening, leading to neuronal death. This study shows how the transition of α-synuclein from its monomeric to oligomeric structure alters its functional consequences in Parkinson's disease.

Description

Keywords

Animals, Coculture Techniques, Embryonic Stem Cells, Humans, Induced Pluripotent Stem Cells, Lipid Peroxidation, Mitochondria, Mitochondrial Membrane Transport Proteins, Mitochondrial Permeability Transition Pore, Mitochondrial Proton-Translocating ATPases, Neurons, Oxidation-Reduction, Parkinson Disease, Patch-Clamp Techniques, Permeability, Proteomics, Rats, Rats, Sprague-Dawley, Reactive Oxygen Species, alpha-Synuclein

Journal Title

Nat Commun

Conference Name

Journal ISSN

2041-1723
2041-1723

Volume Title

9

Publisher

Springer Science and Business Media LLC