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Int J Mol Sci ; 23(19)2022 Sep 27.
Article in English | MEDLINE | ID: mdl-36232716

ABSTRACT

The α-synucleinopathies constitute a subset of neurodegenerative disorders, of which Parkinson's disease (PD) is the most common worldwide, characterized by the accumulation of misfolded α-synuclein in the cytoplasm of neurons, which spreads in a prion-like manner to anatomically interconnected brain areas. However, it is not clear how α-synucleinopathy triggers neurodegeneration. We recently developed a rat model through a single intranigral administration of the neurotoxic ß-sitosterol ß-D-glucoside (BSSG), which produces α-synucleinopathy. In this model, we aimed to evaluate the temporal pattern of levels in oxidative and nitrosative stress and mitochondrial complex I (CI) dysfunction and how these biochemical parameters are associated with neurodegeneration in different brain areas with α-synucleinopathy (Substantia nigra pars compacta, the striatum, in the hippocampus and the olfactory bulb, where α-syn aggregation spreads). Interestingly, an increase in oxidative stress and mitochondrial CI dysfunction accompanied neurodegeneration in those brain regions. Furthermore, in silico analysis suggests a high-affinity binding site for BSSG with peroxisome proliferator-activated receptors (PPAR) alpha (PPAR-α) and gamma (PPAR-γ). These findings will contribute to elucidating the pathophysiological mechanisms associated with α-synucleinopathies and lead to the identification of new early biomarkers and therapeutic targets.


Subject(s)
Brain , Electron Transport Complex I , Mitochondria , Oxidative Stress , Synucleinopathies , alpha-Synuclein , Animals , Brain/metabolism , Brain/physiopathology , Disease Models, Animal , Electron Transport Complex I/metabolism , Mitochondria/metabolism , Nitrosative Stress , Peroxisome Proliferator-Activated Receptors/metabolism , Rats , Synucleinopathies/metabolism , Synucleinopathies/physiopathology , alpha-Synuclein/chemistry , alpha-Synuclein/metabolism
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