Abstract
Mitochondria are essential for neuronal survival and function, and mitochondrial dysfunction plays a critical role in the pathological development of Parkinson's disease (PD). Mitochondrial quality control is known to contribute to the survival of dopaminergic (DA) neurons, with mitophagy being a key regulator of the quality control system. In this study, we show that mitophagy is impaired in the substantia nigra pars compacta (SNc) of the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced mouse model of PD. Treatment with the sigma-1 receptor (Sig 1R) agonist 2-morpholin-4-ylethyl 1-phenylcyclohexane-1-carboxylate (PRE-084) reduced loss of DA neurons, restored motor ability and MPTP-induced damage to mitophagy activity in the SNc of PD-like mice. Additionally, knockdown of Sig 1R in SH-SY5Y DA cells inhibited mitophagy and enhanced 1-methyl-4-phenylpyridinium ion (MPP+) neurotoxicity, whereas application of the Sig 1R selective agonist SKF10047 promoted clearance of damaged mitochondria. Moreover, knockdown of Sig 1R in SH-SY5Y cells resulted in decreased levels of p-ULK1 (Unc-51 Like Autophagy Activating Kinase 1) (Ser555), p-TBK1 (TANK Binding Kinase 1) (Ser172), p-ubiquitin (Ub) (Ser65), Parkin recruitment, and stabilization of PTEN-induced putative kinase 1 (PINK1) in mitochondria. The present data provide the first evidence for potential roles of PINK1/Parkin in Sig 1R-modulated mitophagy in DA neurons.
Keywords:
Mitophagy; PD; PINK1/Parkin; PRE-084; Sigma-1 receptor.
Copyright © 2020 Elsevier Ltd. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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1-Methyl-4-phenylpyridinium / toxicity
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Animals
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Autophagy-Related Protein-1 Homolog / drug effects
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Autophagy-Related Protein-1 Homolog / metabolism
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Cell Line
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Dopaminergic Neurons / drug effects
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Dopaminergic Neurons / metabolism*
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Gene Knockdown Techniques
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Mice
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Mitochondria / drug effects
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Mitochondria / metabolism*
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Mitophagy / drug effects
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Mitophagy / genetics*
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Morpholines / pharmacology
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Parkinsonian Disorders / genetics
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Parkinsonian Disorders / metabolism*
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Parkinsonian Disorders / pathology
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Pars Compacta / drug effects
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Pars Compacta / metabolism
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Pars Compacta / pathology
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Phenazocine / analogs & derivatives
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Phenazocine / pharmacology
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Phosphorylation
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Protein Kinases / drug effects
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Protein Kinases / metabolism*
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Protein Serine-Threonine Kinases / drug effects
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Protein Serine-Threonine Kinases / metabolism
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Protein Stability / drug effects
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Protein Transport / drug effects
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Receptors, sigma / agonists
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Receptors, sigma / genetics*
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Receptors, sigma / metabolism
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Sigma-1 Receptor
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Signal Transduction
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Substantia Nigra / drug effects
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Substantia Nigra / metabolism
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Substantia Nigra / pathology
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Ubiquitin / drug effects
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Ubiquitin / metabolism
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Ubiquitin-Protein Ligases / drug effects
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Ubiquitin-Protein Ligases / metabolism*
Substances
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Morpholines
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Receptors, sigma
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Ubiquitin
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2-(4-morpholino)ethyl-1-phenylcyclohexane-1-carboxylate
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SK&F 10047
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Ubiquitin-Protein Ligases
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parkin protein
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Protein Kinases
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Tbk1 protein, mouse
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Autophagy-Related Protein-1 Homolog
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PTEN-induced putative kinase
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Protein Serine-Threonine Kinases
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Ulk1 protein, mouse
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Phenazocine
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1-Methyl-4-phenylpyridinium