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

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The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease is …
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scholarly articleQ13442814

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P356DOI10.1016/J.NBD.2016.10.006
P932PMC publication ID5214572
P698PubMed publication ID27818324

P50authorMichael R. HaydenQ6833706
P2093author name stringJun Wu
Ilya Bezprozvanny
Iris Grossman
Rebecca Kusko
Michal Geva
Daniel Ryskamp
P2860cites workThe dopamine stabilizers ACR16 and (−)-OSU6162 display nanomolar affinities at the σ-1 receptorQ60049913
The sigma-1 receptor enhances brain plasticity and functional recovery after experimental strokeQ60597042
Sigma Receptor Type 1 Gene Variation in a Group of Polish Patients with Alzheimer’s Disease and Mild Cognitive ImpairmentQ61945786
Dendritic and axonal targeting of type 5 metabotropic glutamate receptor is regulated by homer1 proteins and neuronal excitationQ73258556
The sigma enigma: in vitro/in silico site-directed mutagenesis studies unveil σ1 receptor ligand bindingQ87753078
The Homer family proteinsQ21184127
Homer binds a novel proline-rich motif and links group 1 metabotropic glutamate receptors with IP3 receptorsQ22007971
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1Q24310249
A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosisQ24322790
Neuroprotective effects of inositol 1,4,5-trisphosphate receptor C-terminal fragment in a Huntington's disease mouse modelQ24647950
Role of sigma-1 receptor C-terminal segment in inositol 1,4,5-trisphosphate receptor activation: constitutive enhancement of calcium signaling in MCF-7 tumor cellsQ24652395
The synaptic maintenance problem: membrane recycling, Ca2+ homeostasis and late onset degenerationQ27000356
Crystal structure of the human σ1 receptorQ27704518
Role of sigma-1 receptors in neurodegenerative diseasesQ28082554
Homer 1b regulates the trafficking of group I metabotropic glutamate receptorsQ28143150
Generation and phenotypic analysis of sigma receptor type I (σ1) knockout miceQ28184848
Agonist-independent activation of metabotropic glutamate receptors by the intracellular protein HomerQ28202988
Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survivalQ28255981
A SIGMAR1 splice-site mutation causes distal hereditary motor neuropathyQ28263528
The scaffold protein Homer1b/c links metabotropic glutamate receptor 5 to extracellular signal-regulated protein kinase cascades in neuronsQ28583632
The sigma-1 receptor is enriched in postsynaptic sites of C-terminals in mouse motoneurons. An anatomical and behavioral studyQ28592425
Automated three-dimensional detection and shape classification of dendritic spines from fluorescence microscopy imagesQ28755127
limma powers differential expression analyses for RNA-sequencing and microarray studiesQ29617988
CRISPR-Cas9 knockin mice for genome editing and cancer modelingQ30607892
Reduced synaptic STIM2 expression and impaired store-operated calcium entry cause destabilization of mature spines in mutant presenilin miceQ33554241
Postsynaptic scaffold protein Homer 1a protects against traumatic brain injury via regulating group I metabotropic glutamate receptorsQ33654169
Expression of inositol trisphosphate receptors.Q33834374
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.Q33850154
Cognition and depression: the effects of fluvoxamine, a sigma-1 receptor agonist, reconsideredQ34108906
Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trialQ34231219
The dopaminergic stabiliser ACR16 counteracts the behavioural primitivization induced by the NMDA receptor antagonist MK-801 in mice: implications for cognitionQ34336118
The sigma-1 receptor chaperone as an inter-organelle signaling modulatorQ34356970
Abnormal motor cortex plasticity in premanifest and very early manifest Huntington diseaseQ34382326
Abnormal motor cortex excitability in preclinical and very early Huntington's diseaseQ34386162
Effects of (-)-OSU6162 and ACR16 on motor activity in rats, indicating a unique mechanism of dopaminergic stabilizationQ34763003
Dendritic spine instability leads to progressive neocortical spine loss in a mouse model of Huntington's disease.Q34910148
The dopaminergic stabilizers pridopidine (ACR16) and (-)-OSU6162 display dopamine D(2) receptor antagonism and fast receptor dissociation propertiesQ35013323
Neuronal store-operated calcium entry pathway as a novel therapeutic target for Huntington's disease treatmentQ35072356
Inhibition of store-operated calcium entry attenuates MPP(+)-induced oxidative stress via preservation of mitochondrial function in PC12 cells: involvement of Homer1aQ35073154
Pharmacological stimulation of sigma-1 receptors has neurorestorative effects in experimental parkinsonismQ35154720
Improved vectors and genome-wide libraries for CRISPR screeningQ35215629
Late-onset inner retinal dysfunction in mice lacking sigma receptor 1 (σR1).Q35247759
Differences in duration of Huntington's disease based on age at onsetQ35449269
Sigma-1 receptor regulates Tau phosphorylation and axon extension by shaping p35 turnover via myristic acidQ35669383
The sigma-1 receptor protects against cellular oxidative stress and activates antioxidant response elementsQ35856904
A perspective on the new mechanism of antidepressants: neuritogenesis through sigma-1 receptorsQ35952491
STIM2 protects hippocampal mushroom spines from amyloid synaptotoxicityQ35954470
Sigma 1 receptor regulates the oxidative stress response in primary retinal Müller glial cells via NRF2 signaling and system xc(-), the Na(+)-independent glutamate-cystine exchangerQ36011646
Scaffolding protein Homer1a protects against NMDA-induced neuronal injuryQ36022288
Neuronal Store-Operated Calcium Entry and Mushroom Spine Loss in Amyloid Precursor Protein Knock-In Mouse Model of Alzheimer's Disease.Q36106832
Enhanced Store-Operated Calcium Entry Leads to Striatal Synaptic Loss in a Huntington's Disease Mouse Model.Q36431970
Neuro(active)steroids actions at the neuromodulatory sigma1 (sigma1) receptor: biochemical and physiological evidences, consequences in neuroprotectionQ36582899
The sigma-1 receptor: roles in neuronal plasticity and diseaseQ36653400
Involvement of unique leucine-zipper motif of PSD-Zip45 (Homer 1c/vesl-1L) in group 1 metabotropic glutamate receptor clusteringQ36702025
PrefaceQ36717105
Sigma1 receptors inhibit store-operated Ca2+ entry by attenuating coupling of STIM1 to Orai1Q36789920
Homer proteins in Ca2+ signaling by excitable and non-excitable cellsQ36873568
In vivo protection against retinal neurodegeneration by sigma receptor 1 ligand (+)-pentazocineQ36925899
A prototypical Sigma-1 receptor antagonist protects against brain ischemiaQ36976939
Insights into the Sigma-1 receptor chaperone's cellular functions: a microarray reportQ36995785
The pharmacology of sigma-1 receptorsQ37445561
Sigma-1 receptors regulate hippocampal dendritic spine formation via a free radical-sensitive mechanism involving Rac1xGTP pathwayQ37471826
Early synaptic pathophysiology in neurodegeneration: insights from Huntington's diseaseQ37789909
Homer proteins in Ca²⁺ entry.Q38095898
Corticostriatal circuit dysfunction in Huntington's disease: intersection of glutamate, dopamine and calciumQ38603960
Pridopidine, a dopamine stabilizer, improves motor performance and shows neuroprotective effects in Huntington disease R6/2 mouse modelQ38861728
Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-κB pathway.Q39148056
Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease.Q39638549
Pridopidine selectively occupies sigma-1 rather than dopamine D2 receptors at behaviorally active dosesQ40753593
Co-expression of metabotropic glutamate receptor type 1alpha with homer-1a/Vesl-1S increases the cell surface expression of the receptorQ40940136
The anti-amnesic and neuroprotective effects of donepezil against amyloid beta25-35 peptide-induced toxicity in mice involve an interaction with the sigma1 receptorQ41603023
Pridopidine activates neuroprotective pathways impaired in Huntington DiseaseQ42324179
Anti-amnesic and neuroprotective actions of the sigma-1 receptor agonist (-)-MR22 in rats with selective cholinergic lesion and amyloid infusion.Q42483575
Genetic analysis of SIGMAR1 as a cause of familial ALS with dementiaQ42791389
Efficacy and safety of the dopaminergic stabilizer Pridopidine (ACR16) in patients with Huntington's diseaseQ42975568
Cocaine inhibits store-operated Ca2+ entry in brain microvascular endothelial cells: critical role for sigma-1 receptorsQ43129017
Solution NMR studies reveal the location of the second transmembrane domain of the human sigma-1 receptor.Q43152603
Low density of sigma1 receptors in early Alzheimer's diseaseQ44014199
SKF-96365 attenuates toxin-induced neuronal injury through opposite regulatory effects on Homer1a and Homer1b/c in cultured rat mesencephalic cellsQ44296254
σ-1 Receptors (σ1Binding Sites) Form Raft-Like Microdomains and Target Lipid Droplets on the Endoplasmic Reticulum: Roles in Endoplasmic Reticulum Lipid Compartmentalization and ExportQ44429451
Antiamnesic and neuroprotective effects of the aminotetrahydrofuran derivative ANAVEX1-41 against amyloid beta(25-35)-induced toxicity in miceQ44617703
Selective striatal neuronal loss in a YAC128 mouse model of Huntington diseaseQ45289366
Huntington disease: Clinical care and evaluationQ45290085
A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's diseaseQ45292205
Calbindin D28K as a marker for the degeneration of the striatonigral pathway in Huntington's diseaseQ45294692
Clinical and neuropathologic assessment of severity in Huntington's diseaseQ45298780
Regional cerebral glucose metabolism after pridopidine (ACR16) treatment in patients with Huntington diseaseQ45302933
Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein loadQ45306738
Anti-amnesic properties of (+/-)-PPCC, a novel sigma receptor ligand, on cognitive dysfunction induced by selective cholinergic lesion in rats.Q46107849
Calbindin d28k overexpression protects striatal neurons from transient focal cerebral ischemiaQ46132048
Neuroprotective effects of sigma-1 receptor agonists against beta-amyloid-induced toxicity.Q46596943
Association between a variant of the sigma-1 receptor gene and Alzheimer's diseaseQ47803612
High Ca2+-phosphate transfection efficiency in low-density neuronal culturesQ48218879
Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration.Q48330721
Huntington diseaseQ48465200
Relative sparing in Parkinson's disease of substantia nigra dopamine neurons containing calbindin-D28K.Q48899006
The presence of calbindin in rat cortical neurons protects in vitro from oxydative stressQ49160218
A variant of the sigma receptor type-1 gene is a protective factor for Alzheimer disease.Q51986787
Investigation of the role of sigma1-receptors in inositol 1,4,5-trisphosphate dependent calcium signaling in hepatocytes.Q54585068
Huntington diseaseQ56083219
P4510describes a project that useslimmaQ112236343
ImageJQ1659584
P433issuePt A
P921main subjectHuntington's diseaseQ190564
P304page(s)46-59
P577publication date2016-11-03
P1433published inNeurobiology of DiseaseQ15716606
P1476titleThe sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease
P478volume97

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cites work (P2860)
Q55438236Altering cortical input unmasks synaptic phenotypes in the YAC128 cortico-striatal co-culture model of Huntington disease.
Q89963647Axonal Endoplasmic Reticulum Dynamics and Its Roles in Neurodegeneration
Q50088379Cause or compensation?-Altered neuronal Ca2+ handling in Huntington's disease.
Q64077579Combinatorial treatment increases IKAP levels in human cells generated from Familial Dysautonomia patients
Q57073756Drug Screening Identifies Sigma-1-Receptor as a Target for the Therapy of VWM Leukodystrophy
Q45305901Early pridopidine treatment improves behavioral and transcriptional deficits in YAC128 Huntington disease mice.
Q92478041Impairment and Restoration of Homeostatic Plasticity in Cultured Cortical Neurons From a Mouse Model of Huntington Disease
Q52430483Inhibition of TRPC1-Dependent Store-Operated Calcium Entry Improves Synaptic Stability and Motor Performance in a Mouse Model of Huntington's Disease.
Q55335504Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse.
Q90679929Mutational Analysis of Sigma-1 Receptor's Role in Synaptic Stability
Q90263450Neuronal Sigma-1 Receptors: Signaling Functions and Protective Roles in Neurodegenerative Diseases
Q47410169Neuroprotective Effect of σ1-Receptors on the Cell Model of Huntington's Disease
Q52721231On the Role of Store-Operated Calcium Entry in Acute and Chronic Neurodegenerative Diseases.
Q92328222Partners in Crime: Towards New Ways of Targeting Calcium Channels
Q52564664Pridopidine Reverses Phencyclidine-Induced Memory Impairment.
Q92158893Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor
Q52685691Pridopidine: Overview of Pharmacology and Rationale for its Use in Huntington's Disease.
Q64079932Protein Misfolding and ER Stress in Huntington's Disease
Q41716404Role of the sigma-1 receptor chaperone in rod and cone photoreceptor degenerations in a mouse model of retinitis pigmentosa
Q41714466Roles of sigma-1 receptors on mitochondrial functions relevant to neurodegenerative diseases
Q67228612Safety and efficacy of pridopidine in patients with Huntington's disease (PRIDE-HD): a phase 2, randomised, placebo-controlled, multicentre, dose-ranging study
Q101226279Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
Q64075421Targeting the Sigma-1 Receptor via Pridopidine Ameliorates Central Features of ALS Pathology in a SOD1 Model
Q50973291The Role of Sigma-1 Receptor, an Intracellular Chaperone in Neurodegenerative Diseases.
Q64859904Therapeutic approaches to Huntington disease: from the bench to the clinic
Q47977953Treatment options for chorea

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