(Q64079932)

April 2022 Public Data File from Crossref

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Differential loss of striatal projection neurons in Huntington disease

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12 December 2020

The carboxy terminus of the beta amyloid protein is critical for the seeding of amyloid formation: implications for the pathogenesis of Alzheimer's disease

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12 December 2020

Purification, molecular cloning, and expression of the mammalian sigma1-binding site

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12 December 2020

Cellular delivery of human CNTF prevents motor and cognitive dysfunction in a rodent model of Huntington's disease

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12 December 2020

Huntington aggregates may not predict neuronal death in Huntington's disease

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12 December 2020

Genetic suppression of polyglutamine toxicity in Drosophila

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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

Understanding protein folding via free-energy surfaces from theory and experiment

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12 December 2020

Feedback inhibition of the unfolded protein response by GADD34-mediated dephosphorylation of eIF2alpha

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12 December 2020

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

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12 December 2020

Molecular chaperones in the cytosol: from nascent chain to folded protein

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12 December 2020

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

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12 December 2020

Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1

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12 December 2020

Toxic proteins in neurodegenerative disease

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12 December 2020

ER stress regulation of ATF6 localization by dissociation of BiP/GRP78 binding and unmasking of Golgi localization signals

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12 December 2020

Peptide aggregation in neurodegenerative disease

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12 December 2020

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

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12 December 2020

Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity

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12 December 2020

Translational control in the endoplasmic reticulum stress response

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12 December 2020

Unfolding the role of protein misfolding in neurodegenerative diseases

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12 December 2020

Intracellular Dynamics of σ-1 Receptors (σ1Binding Sites) in NG108-15 Cells

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12 December 2020

Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

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12 December 2020

Protein folding and misfolding: a paradigm of self-assembly and regulation in complex biological systems

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12 December 2020

Nrf2 Is a Direct PERK Substrate and Effector of PERK-Dependent Cell Survival

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12 December 2020

Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

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12 December 2020

Roles of CHOP/GADD153 in endoplasmic reticulum stress

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12 December 2020

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

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12 December 2020

Protein aggregation and neurodegenerative disease

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12 December 2020

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

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12 December 2020

The biological and chemical basis for tissue-selective amyloid disease

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12 December 2020

The unfolded protein response is activated in Alzheimer's disease.

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12 December 2020

Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery: a long-term follow-up study

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12 December 2020

Protein aggregation in crowded environments.

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12 December 2020

Sigma 1 receptor agonists act as neuroprotective drugs through inhibition of inducible nitric oxide synthase.

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12 December 2020

Sigma-1 receptor activation prevents intracellular calcium dysregulation in cortical neurons during in vitro ischemia

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12 December 2020

The anti-amnesic and neuroprotective effects of donepezil against amyloid beta25-35 peptide-induced toxicity in mice involve an interaction with the sigma1 receptor

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12 December 2020

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12 December 2020

Is the ubiquitin-proteasome system impaired in Huntington's disease?

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12 December 2020

Global changes to the ubiquitin system in Huntington's disease.

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12 December 2020

Polyglutamine diseases: emerging concepts in pathogenesis and therapy

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12 December 2020

Location, location, location: altered transcription factor trafficking in neurodegeneration

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12 December 2020

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

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12 December 2020

Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survival

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12 December 2020

Plaque-bearing mice with reduced levels of oligomeric amyloid-beta assemblies have intact memory function.

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12 December 2020

Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins

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12 December 2020

The ubiquitin-proteasome pathway in Huntington's disease.

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12 December 2020

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

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12 December 2020

Protein aggregation kinetics, mechanism, and curve-fitting: a review of the literature.

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12 December 2020

Amyloid formation by globular proteins under native conditions

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12 December 2020

MAM: more than just a housekeeper

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12 December 2020

SCAMP5 links endoplasmic reticulum stress to the accumulation of expanded polyglutamine protein aggregates via endocytosis inhibition

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12 December 2020

The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease

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12 December 2020

Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease

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12 December 2020

Mechanisms of protein aggregation

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12 December 2020

Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease

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12 December 2020

Role of ERO1-alpha-mediated stimulation of inositol 1,4,5-triphosphate receptor activity in endoplasmic reticulum stress-induced apoptosis

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12 December 2020

Sigma-1 receptors regulate Bcl-2 expression by reactive oxygen species-dependent transcriptional regulation of nuclear factor kappaB

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12 December 2020

Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP.

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12 December 2020

Embryonic striatal grafts restore bi-directional synaptic plasticity in a rodent model of Huntington's disease

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12 December 2020

Protein homeostasis and aging: The importance of exquisite quality control

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12 December 2020

Ero1alpha requires oxidizing and normoxic conditions to localize to the mitochondria-associated membrane (MAM).

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12 December 2020

Protein homeostasis and aging in neurodegeneration

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12 December 2020

The sigma-1 receptor chaperone as an inter-organelle signaling modulator

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12 December 2020

Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease

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12 December 2020

Formation and toxicity of soluble polyglutamine oligomers in living cells

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12 December 2020

Selective inhibition of a regulatory subunit of protein phosphatase 1 restores proteostasis

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12 December 2020

Aging as an event of proteostasis collapse

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12 December 2020

Protein aggregate spreading in neurodegenerative diseases: problems and perspectives

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12 December 2020

A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis

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12 December 2020

Neuronal circuitry regulates the response of Caenorhabditis elegans to misfolded proteins

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12 December 2020

New insights into the role of mitochondria-associated endoplasmic reticulum membrane

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12 December 2020

Sigma-1Rs are upregulated via PERK/eIF2α/ATF4 pathway and execute protective function in ER stress.

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12 December 2020

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

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12 December 2020

Mechanism-based screen for G1/S checkpoint activators identifies a selective activator of EIF2AK3/PERK signalling

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12 December 2020

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

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12 December 2020

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease

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12 December 2020

The sigma-1 receptor protects against cellular oxidative stress and activates antioxidant response elements

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12 December 2020

The amyloid state of proteins in human diseases

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12 December 2020

Sigma-1R agonist improves motor function and motoneuron survival in ALS mice

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12 December 2020

Intracellular accumulation of toxic turn amyloid-β is associated with endoplasmic reticulum stress in Alzheimer's disease

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12 December 2020

Sigma 1 receptor stimulation protects against oxidative damage through suppression of the ER stress responses in the human lens.

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12 December 2020

The sigma-1 receptor: roles in neuronal plasticity and disease

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12 December 2020

Mild cognitive impairment due to Alzheimer disease in the community

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12 December 2020

Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-κB pathway.

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12 December 2020

Tau accumulation activates the unfolded protein response by impairing endoplasmic reticulum-associated degradation

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12 December 2020

Pharmacological brake-release of mRNA translation enhances cognitive memory

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12 December 2020

ER stress-induced cell death mechanisms

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12 December 2020

IRE1: ER stress sensor and cell fate executor

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12 December 2020

Donor age dependent graft development and recovery in a rat model of Huntington's disease: histological and behavioral analysis.

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12 December 2020

Stimulation of sigma receptors with afobazole blocks activation of microglia and reduces toxicity caused by amyloid-β25-35.

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12 December 2020

Oral treatment targeting the unfolded protein response prevents neurodegeneration and clinical disease in prion-infected mice

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12 December 2020

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

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12 December 2020

The unfolded protein response is activated in disease-affected brain regions in progressive supranuclear palsy and Alzheimer's disease

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12 December 2020

The ubiquitin-proteasome system in neurodegeneration

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12 December 2020

Loss of proteostasis induced by amyloid beta peptide in brain endothelial cells

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12 December 2020

ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin

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12 December 2020

Barcoding heat shock proteins to human diseases: looking beyond the heat shock response

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12 December 2020

Pharmacological stimulation of sigma-1 receptors has neurorestorative effects in experimental parkinsonism

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12 December 2020

Proteostasis impairment in protein-misfolding and -aggregation diseases

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12 December 2020

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1α.

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12 December 2020

Sigma receptors as potential therapeutic targets for neuroprotection

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12 December 2020

Sigma-1 receptor chaperones in neurodegenerative and psychiatric disorders

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12 December 2020

Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity?

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12 December 2020

A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease

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12 December 2020

Sigma-1 receptor is involved in degradation of intranuclear inclusions in a cellular model of Huntington's disease

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12 December 2020

Differential regulation of the heat shock factor 1 and DAF-16 by neuronal nhl-1 in the nematode C. elegans.

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12 December 2020

A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits

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12 December 2020

Solution NMR studies reveal the location of the second transmembrane domain of the human sigma-1 receptor.

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12 December 2020

Compartment-specific aggregases direct distinct nuclear and cytoplasmic aggregate deposition

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12 December 2020

Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration.

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12 December 2020

Sigma-1 receptor: the novel intracellular target of neuropsychotherapeutic drugs

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12 December 2020

Role of sigma-1 receptors in neurodegenerative diseases

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12 December 2020

Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity

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12 December 2020

The biology of proteostasis in aging and disease

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12 December 2020

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

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12 December 2020

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12 December 2020

Pridopidine, a dopamine stabilizer, improves motor performance and shows neuroprotective effects in Huntington disease R6/2 mouse model

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12 December 2020

NEURODEGENERATION. Alzheimer's and Parkinson's diseases: The prion concept in relation to assembled Aβ, tau, and α-synuclein

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12 December 2020

Guanabenz Treatment Accelerates Disease in a Mutant SOD1 Mouse Model of ALS.

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12 December 2020

Caenorhabditis elegans DAF-16/FOXO transcription factor and its mammalian homologs associate with age-related disease

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12 December 2020

PERK inhibition prevents tau-mediated neurodegeneration in a mouse model of frontotemporal dementia

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12 December 2020

Profile of pridopidine and its potential in the treatment of Huntington disease: the evidence to date

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12 December 2020

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.

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12 December 2020

ENC1 Modulates the Aggregation and Neurotoxicity of Mutant Huntingtin Through p62 Under ER Stress.

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12 December 2020

Gene therapy by proteasome activator, PA28γ, improves motor coordination and proteasome function in Huntington's disease YAC128 mice

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12 December 2020

Protein aggregation and ER stress

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12 December 2020

Endoplasmic reticulum stress: The cause and solution to Huntington's disease?

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12 December 2020

Crystal structure of the human σ1 receptor

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12 December 2020

Repression of the eIF2α kinase PERK alleviates mGluR-LTD impairments in a mouse model of Alzheimer's disease

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12 December 2020

Heat shock proteins as potential targets for protective strategies in neurodegeneration

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12 December 2020

The Role of Amyloid-β Oligomers in Toxicity, Propagation, and Immunotherapy

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12 December 2020

In vivo aspects of protein folding and quality control

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12 December 2020

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease

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12 December 2020

Huntington's disease: Molecular basis of pathology and status of current therapeutic approaches

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12 December 2020

A new target for Parkinson's disease: Small molecule SERCA activator CDN1163 ameliorates dyskinesia in 6-OHDA-lesioned rats.

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12 December 2020

The role of endoplasmic reticulum stress in neurodegenerative disease

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12 December 2020

The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease

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12 December 2020

Is there a place for human fetal-derived stem cells for cell replacement therapy in Huntington's disease?

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12 December 2020

PERK activation mitigates tau pathology in vitro and in vivo

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12 December 2020

The expanded CAG repeat in the huntingtin gene as target for therapeutic RNA modulation throughout the HD mouse brain.

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12 December 2020

Sigma-1 Receptors and Neurodegenerative Diseases: Towards a Hypothesis of Sigma-1 Receptors as Amplifiers of Neurodegeneration and Neuroprotection

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12 December 2020

Is Huntingtin Dispensable in the Adult Brain?

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12 December 2020

Attenuation of polyglutamine-induced toxicity by enhancement of mitochondrial OXPHOS in yeast and fly models of aging.

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12 December 2020

Repurposed drugs targeting eIF2α-P-mediated translational repression prevent neurodegeneration in mice

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12 December 2020

Protein Misfolding Diseases.

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12 December 2020

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

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12 December 2020

Huntingtin Inclusions Trigger Cellular Quiescence, Deactivate Apoptosis, and Lead to Delayed Necrosis

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12 December 2020

Protein Misfolding, Amyloid Formation, and Human Disease: A Summary of Progress Over the Last Decade

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12 December 2020

CRISPR/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington's disease

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12 December 2020

Metformin intake associates with better cognitive function in patients with Huntington's disease.

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12 December 2020

The stress response factor daf-16/FOXO is required for multiple compound families to prolong the function of neurons with Huntington's disease

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12 December 2020

Fine-tuning PERK signaling for neuroprotection.

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12 December 2020

The Endoplasmic Reticulum Unfolded Protein Response in Neurodegenerative Disorders and Its Potential Therapeutic Significance

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12 December 2020

Huntington's Disease: Pathogenic Mechanisms and Therapeutic Targets.

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12 December 2020

A selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington's disease mice

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12 December 2020

Transcriptional profiles for distinct aggregation states of mutant Huntingtin exon 1 protein unmask new Huntington's disease pathways

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12 December 2020

A review on protein misfolding, aggregation and strategies to prevent related ailments

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12 December 2020

AAV5-miHTT gene therapy demonstrates suppression of mutant huntingtin aggregation and neuronal dysfunction in a rat model of Huntington's disease.

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12 December 2020

PERK as a hub of multiple pathogenic pathways leading to memory deficits and neurodegeneration in Alzheimer's disease.

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12 December 2020

The neuroprotective transcription factor ATF5 is decreased and sequestered into polyglutamine inclusions in Huntington's disease

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12 December 2020

APEX2- tagging of Sigma 1-receptor indicates subcellular protein topology with cytosolic N-terminus and ER luminal C-terminus

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12 December 2020

Protein phosphatase 1 regulates huntingtin exon 1 aggregation and toxicity.

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12 December 2020

Alpha-synuclein induces the unfolded protein response in Parkinson's disease SNCA triplication iPSC-derived neurons

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12 December 2020

The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin.

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12 December 2020

The Unfolded Protein Response and Cell Fate Control

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12 December 2020

Recent insights into PERK-dependent signaling from the stressed endoplasmic reticulum

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12 December 2020

Neuroprotective Effect of σ1-Receptors on the Cell Model of Huntington's Disease

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12 December 2020

RNAi mechanisms in Huntington's disease therapy: siRNA versus shRNA.

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12 December 2020

Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex

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12 December 2020

Early pridopidine treatment improves behavioral and transcriptional deficits in YAC128 Huntington disease mice.

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12 December 2020

(off)Targeting UPR signaling: the race toward intervening ER proteostasis

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12 December 2020

The 17-residue-long N terminus in huntingtin controls step-wise aggregation in solution and on membranes via different mechanisms

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12 December 2020

Mechanisms of disordered neurodegenerative function: concepts and facts about the different roles of the protein kinase RNA-like endoplasmic reticulum kinase (PERK).

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12 December 2020

Cellular Handling of Protein Aggregates by Disaggregation Machines.

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12 December 2020

Targeting PERK signaling with the small molecule GSK2606414 prevents neurodegeneration in a model of Parkinson's disease

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12 December 2020

Herp Promotes Degradation of Mutant Huntingtin: Involvement of the Proteasome and Molecular Chaperones.

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12 December 2020

Guanabenz promotes neuronal survival via enhancement of ATF4 and parkin expression in models of Parkinson disease.

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12 December 2020

Correction of Huntington's Disease Phenotype by Genistein-Induced Autophagy in the Cellular Model.

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12 December 2020

Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease.

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12 December 2020

The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.

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12 December 2020

Inhibition of TRPC1-Dependent Store-Operated Calcium Entry Improves Synaptic Stability and Motor Performance in a Mouse Model of Huntington's Disease.

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12 December 2020

Pridopidine: Overview of Pharmacology and Rationale for its Use in Huntington's Disease.

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12 December 2020

Precise Excision of the CAG Tract from the Huntingtin Gene by Cas9 Nickases.

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12 December 2020

Huntington's disease: novel therapeutic perspectives hanging in the balance.

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12 December 2020

Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse.

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12 December 2020

AAV5-miHTT Gene Therapy Demonstrates Broad Distribution and Strong Human Mutant Huntingtin Lowering in a Huntington's Disease Minipig Model

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12 December 2020

Huntington's disease: the coming of age

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12 December 2020

Tauopathy-associated PERK alleles are functional hypomorphs that increase neuronal vulnerability to ER stress

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12 December 2020

Could a Common Mechanism of Protein Degradation Impairment Underlie Many Neurodegenerative Diseases?

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12 December 2020

Metformin reverses early cortical network dysfunction and behavior changes in Huntington's disease

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12 December 2020

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington’s Disease

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12 December 2020

Therapeutic approaches to Huntington disease: from the bench to the clinic

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12 December 2020

Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases

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12 December 2020

Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease

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12 December 2020

A Mitochondria-Associated Oxidative Stress Perspective on Huntington's Disease

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12 December 2020