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Adapting the endoplasmic reticulum proteostasis rescues epilepsy-associated NMDA receptor variants
The GRIN genes encoding N -methyl- D -aspartate receptor (NMDAR) subunits are remarkably intolerant to variation. Many pathogenic NMDAR variants result...
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Proteostasis failure exacerbates neuronal circuit dysfunction and sleep impairments in Alzheimer’s disease
Failed proteostasis is a well-documented feature of Alzheimer’s disease, particularly, reduced protein degradation and clearance. However, the...
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Methods for In Vivo Characterization of Proteostasis in the Mouse Retina
An increasing number of studies connect inherited and age-related retinal degenerations with changes in the regulation of proteostasis. Here, we...
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Broad proteomics analysis of seeding-induced aggregation of α-synuclein in M83 neurons reveals remodeling of proteostasis mechanisms that might contribute to Parkinson’s disease pathogenesis
Aggregation of misfolded α-synuclein (α-syn) is a key characteristic feature of Parkinson’s disease (PD) and related synucleinopathies. The nature of...
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Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons
BackgroundProgranulin (PGRN) is a lysosomal glycoprotein implicated in various neurodegenerative diseases, including frontotemporal dementia and...
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Improper Proteostasis: Can It Serve as Biomarkers for Neurodegenerative Diseases?
Cells synthesize new proteins after multiple molecular decisions. Damage of existing proteins, accumulation of abnormal proteins, and basic...
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Regulators of proteostasis are translationally repressed in fibroblasts from patients with sporadic and LRRK2-G2019S Parkinson’s disease
Deficits in protein synthesis are associated with Parkinson’s disease (PD). However, it is not known which proteins are affected or if there are...
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DOPAL initiates αSynuclein-dependent impaired proteostasis and degeneration of neuronal projections in Parkinson’s disease
Dopamine dyshomeostasis has been acknowledged among the determinants of nigrostriatal neuron degeneration in Parkinson’s disease (PD). Several...
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Functional genomics screen identifies proteostasis targets that modulate prion protein (PrP) stability
Prion protein (PrP) adopts either a helical conformation (PrP C ) or an alternative, beta sheet-rich, misfolded conformation (PrP Sc ). The PrP Sc form...
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Effects of Cell Proteostasis Network on the Survival of SARS-CoV-2
The proteostasis network includes all the factors that control the function of proteins in their native state and minimize their non-functional or...
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Mitochondrial DNA variants modulate N-formylmethionine, proteostasis and risk of late-onset human diseases
Mitochondrial DNA (mtDNA) variants influence the risk of late-onset human diseases, but the reasons for this are poorly understood. Undertaking a...
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Chronic Cerebral Hypoperfusion-Induced Disturbed Proteostasis of Mitochondria and MAM Is Reflected in the CSF of Rats by Proteomic Analysis
Declining cerebral blood flow leads to chronic cerebral hypoperfusion which can induce neurodegenerative disorders, such as vascular dementia. The...
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Flotillin-1 palmitoylation turnover by APT-1 and ZDHHC-19 promotes cervical cancer progression by suppressing IGF-1 receptor desensitization and proteostasis
We have shown that insulin-like growth factor-1 (IGF-1) induces palmitoylation turnover of Flotillin-1 (Flot-1) in the plasma membrane (PM) for cell...
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Multi-platform proteomic analysis of Alzheimer’s disease cerebrospinal fluid and plasma reveals network biomarkers associated with proteostasis and the matrisome
Robust and accessible biomarkers that can capture the heterogeneity of Alzheimer’s disease and its diverse pathological processes are urgently...
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Endoplasmic Reticulum in Metaplasticity: From Information Processing to Synaptic Proteostasis
The ER (endoplasmic reticulum) is a Ca 2+ reservoir and the unique protein-synthesizing machinery which is distributed throughout the neuron and...
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Peroxiredoxin 6 mediates protective function of astrocytes in Aβ proteostasis
BackgroundDisruption of β-amyloid (Aβ) homeostasis is the initial culprit in Alzheimer’s disease (AD) pathogenesis. Astrocytes respond to emerging Aβ...
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The functions and regulation of heat shock proteins; key orchestrators of proteostasis and the heat shock response
Cells respond to protein-damaging (proteotoxic) stress by activation of the Heat Shock Response (HSR). The HSR provides cells with an enhanced...
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High-intensity interval training alleviates exhaustive exercise-induced HSP70-assisted selective autophagy in skeletal muscle
This study was designed to probe the effect of chaperone-assisted selective autophagy (CASA) on the maintenance of proteostasis during exhaustive...
