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

Amyloid-β Peptides are Generated in Mitochondria-Associated Endoplasmic Reticulum Membranes

(2018) Bernadette Schreiner et al.   JOURNAL OF ALZHEIMERS DISEASE

From dysfunctional endoplasmic reticulum-mitochondria coupling to neurodegeneration

(2017) Zoi Erpapazoglou et al.   NEUROCHEMISTRY INTERNATIONAL

Blockade of RyRs in the ER Attenuates 6-OHDA-Induced Calcium Overload, Cellular Hypo-Excitability and Apoptosis in Dopaminergic Neurons

(2017) Lu Huang et al.   Frontiers in Cellular Neuroscience

The Endoplasmic Reticulum Chaperone GRP78/BiP Modulates Prion Propagation in vitro and in vivo

(2017) Kyung-Won Park et al.   Scientific Reports

Mitochondria-associated membranes as hubs for neurodegeneration

(2016) Michiel Krols et al.   ACTA NEUROPATHOLOGICA

Identification of a microRNA (miR-663a) induced by ER stress and its target gene PLOD3 by a combined microRNome and proteome approach

(2016) Giuseppina Amodio et al.   CELL BIOLOGY AND TOXICOLOGY

Protein Folding and Misfolding, Endoplasmic Reticulum Stress in Neurodegenerative Diseases: in Trace of Novel Drug Targets

(2016) Botond Penke et al.   CURRENT PROTEIN & PEPTIDE SCIENCE

Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death

(2016) Chantal Sellier et al.   EMBO JOURNAL

Progesterone exerts neuroprotective effects against Aβ-induced neuroinflammation by attenuating ER stress in astrocytes

(2016) Yang Hong et al.   INTERNATIONAL IMMUNOPHARMACOLOGY

Rescue of Glaucomatous Neurodegeneration by Differentially Modulating Neuronal Endoplasmic Reticulum Stress Molecules

(2016) L. Yang et al.   JOURNAL OF NEUROSCIENCE

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

(2016) Wenzhong Yang et al.   NEUROBIOLOGY OF AGING

β-Asarone Inhibits IRE1/XBP1 Endoplasmic Reticulum Stress Pathway in 6-OHDA-Induced Parkinsonian Rats

(2016) Baile Ning et al.   NEUROCHEMICAL RESEARCH

Stromal Interaction Molecule 1 rescues store-operated calcium entry and protects NG115-401L cells against cell death induced by endoplasmic reticulum and mitochondrial oxidative stress

(2016) Changfeng Zhang et al.   NEUROCHEMISTRY INTERNATIONAL

Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis

(2016) Sebum Lee et al.   NEURON

Up-regulation of activating transcription factor 4 induces severe loss of dopamine nigral neurons in a rat model of Parkinson’s disease

(2016) Joseph C. Gully et al.   NEUROSCIENCE LETTERS

C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Demen

(2016) Ruxandra Dafinca et al.   STEM CELLS

Mitofusin-mediated ER stress triggers neurodegeneration in pink1/parkin models of Parkinson’s disease

(2016) I Celardo et al.   Cell Death & Disease

ER Stress and Autophagic Perturbations Lead to Elevated Extracellular α-Synuclein in GBA-N370S Parkinson's iPSC-Derived Dopamine Neurons

(2016) Hugo J.R. Fernandes et al.   Stem Cell Reports

Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and α-Synuclein Accumulation

(2016) Sun Young Chung et al.   Stem Cell Reports

Parkinson disease-linked GBA mutation effects reversed by molecular chaperones in human cell and fly models

(2016) Alvaro Sanchez-Martinez et al.   Scientific Reports

The unfolded protein response in neurodegenerative diseases: a neuropathological perspective

(2015) Wiep Scheper et al.   ACTA NEUROPATHOLOGICA

Dysfunction of Endoplasmic Reticulum (ER) and Mitochondria (MT) in Alzheimer's Disease: The Role of the ER-MT Cross-Talk

(2015) Katalin Volgyi et al.   Current Alzheimer Research

Allicin improves endoplasmic reticulum stress-related cognitive deficits via PERK/Nrf2 antioxidative signaling pathway

(2015) Yao-Feng Zhu et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Early Neurodegeneration in the Brain of a Child Without Functional PKR-like Endoplasmic Reticulum Kinase

(2015) Julius Bruch et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

(2015) Huikyong Lee et al.   MOLECULAR NEUROBIOLOGY

Compromised autophagy and neurodegenerative diseases

(2015) Fiona M. Menzies et al.   NATURE REVIEWS NEUROSCIENCE

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

(2015) Fernando G. Vieira et al.   PLoS One

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

(2015) I. Das et al.   SCIENCE

Phenotypic assays identify azoramide as a small-molecule modulator of the unfolded protein response with antidiabetic activity

(2015) Suneng Fu et al.   Science Translational Medicine

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

(2015) M Halliday et al.   Cell Death & Disease

To Be or Not to Be? How Selective Autophagy and Cell Death Govern Cell Fate

(2014) Douglas R. Green et al.   CELL

Mechanisms of protein-folding diseases at a glance

(2014) J. S. Valastyan et al.   Disease Models & Mechanisms

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

(2014) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

The amyloid state and its association with protein misfolding diseases

(2014) Tuomas P. J. Knowles et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

Targeting autophagy in neurodegenerative diseases

(2014) René L. Vidal et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Loss of function of the ALS protein SigR1 leads to ER pathology associated with defective autophagy and lipid raft disturbances

(2014) J T Vollrath et al.   Cell Death & Disease

Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology

(2013) Elisa Teyssou et al.   ACTA NEUROPATHOLOGICA

TNF-α Mediates PKR-Dependent Memory Impairment and Brain IRS-1 Inhibition Induced by Alzheimer’s β-Amyloid Oligomers in Mice and Monkeys

(2013) Mychael V. Lourenco et al.   Cell Metabolism

XBP-1u suppresses autophagy by promoting the degradation of FoxO1 in cancer cells

(2013) Ying Zhao et al.   CELL RESEARCH

Polymorphism −116C/G of Human X-box-Binding Protein 1 Promoter is Associated with Risk of Alzheimer's Disease

(2013) Sheng-Yuan Liu et al.   CNS Neuroscience & Therapeutics

The ALS8 protein VAPB interacts with the ER–Golgi recycling protein YIF1A and regulates membrane delivery into dendrites

(2013) Marijn Kuijpers et al.   EMBO JOURNAL

Endoplasmic Reticulum stress reduces COPII vesicle formation and modifies Sec23a cycling at ERESs

(2013) Giuseppina Amodio et al.   FEBS LETTERS

Association studies indicate that protein disulfide isomerase is a risk factor in amyotrophic lateral sclerosis

(2013) Chun Tak Kwok et al.   FREE RADICAL BIOLOGY AND MEDICINE

The amyotrophic lateral sclerosis 8 protein, VAP, is required for ER protein quality control

(2013) Amina Moustaqim-Barrette et al.   HUMAN MOLECULAR GENETICS

ER-stress-associated functional link between Parkin and DJ-1 via a transcriptional cascade involving the tumor suppressor p53 and the spliced X-box binding protein XBP-1

(2013) E. Duplan et al.   JOURNAL OF CELL SCIENCE

The ire-1 ER stress-response pathway is required for normal secretory-protein metabolism in C. elegans

(2013) M. Safra et al.   JOURNAL OF CELL SCIENCE

Tau Accumulation Activates the Unfolded Protein Response by Impairing Endoplasmic Reticulum-Associated Degradation

(2013) J. F. Abisambra et al.   JOURNAL OF NEUROSCIENCE

Establishing Links between Endoplasmic Reticulum-Mediated Hormesis and Cancer

(2013) B. Mollereau   MOLECULAR AND CELLULAR BIOLOGY

Paneth cells as a site of origin for intestinal inflammation

(2013) Timon E. Adolph et al.   NATURE

Suppression of eIF2α kinases alleviates Alzheimer's disease–related plasticity and memory deficits

(2013) Tao Ma et al.   NATURE NEUROSCIENCE

Targeting the unfolded protein response in disease

(2013) Claudio Hetz et al.   NATURE REVIEWS DRUG DISCOVERY

The eIF2α/ATF4 pathway is essential for stress-induced autophagy gene expression

(2013) Wafa B’chir et al.   NUCLEIC ACIDS RESEARCH

Functional Contribution of the Transcription Factor ATF4 to the Pathogenesis of Amyotrophic Lateral Sclerosis

(2013) Soledad Matus et al.   PLoS One

ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation

(2013) Adam K. Walker et al.   PLoS One

Deletion of the eIF2α Kinase GCN2 Fails to Rescue the Memory Decline Associated with Alzheimer’s Disease

(2013) Latha Devi et al.   PLoS One

Oral Treatment Targeting the Unfolded Protein Response Prevents Neurodegeneration and Clinical Disease in Prion-Infected Mice

(2013) J. A. Moreno et al.   Science Translational Medicine

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

Insights on altered mitochondrial function and dynamics in the pathogenesis of neurodegeneration

(2013) Joseph McInnes   Translational Neurodegeneration

ER stress inhibits neuronal death by promoting autophagy

(2012) Antoine Fouillet et al.   Autophagy

Hormesis

(2012) Soledad Matus et al.   Autophagy

Crosstalk between the UPR and autophagy pathway contributes to handling cellular stress in neurodegenerative disease

(2012) René L. Vidal et al.   Autophagy

Transmissible Proteins: Expanding the Prion Heresy

(2012) Claudio Soto   CELL

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

(2012) Rene L. Vidal et al.   HUMAN MOLECULAR GENETICS

Neurotoxin-induced ER stress in mouse dopaminergic neurons involves downregulation of TRPC1 and inhibition of AKT/mTOR signaling

(2012) Senthil Selvaraj et al.   JOURNAL OF CLINICAL INVESTIGATION

Accumulation of Toxic  -Synuclein Oligomer within Endoplasmic Reticulum Occurs in  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Endoplasmic Reticulum Stress Is Important for the Manifestations of  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Endoplasmic reticulum dysfunction in neurological disease

(2012) Benoit D Roussel et al.   LANCET NEUROLOGY

Glucose Regulated Protein 78 Diminishes α-Synuclein Neurotoxicity in a Rat Model of Parkinson Disease

(2012) Marina S Gorbatyuk et al.   MOLECULAR THERAPY

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

Autophagy modulation as a potential therapeutic target for diverse diseases

(2012) David C. Rubinsztein et al.   NATURE REVIEWS DRUG DISCOVERY

STIM proteins: dynamic calcium signal transducers

(2012) Jonathan Soboloff et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

(2012) Claudio Hetz   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase

(2012) Manal A. Farg et al.   NEUROBIOLOGY OF AGING

JNK3 Perpetuates Metabolic Stress Induced by Aβ Peptides

(2012) Sung Ok Yoon et al.   NEURON

ATF6alpha Promotes Astroglial Activation and Neuronal Survival in a Chronic Mouse Model of Parkinson’s Disease

(2012) Koji Hashida et al.   PLoS One

Endoplasmic reticulum stress and prion diseases

(2012) Kun Xu et al.   REVIEWS IN THE NEUROSCIENCES

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

(2011) Amr Al-Saif et al.   ANNALS OF NEUROLOGY

Recent Advances in the Genetics of Parkinson's Disease

(2011) Ian Martin et al.   Annual Review of Genomics and Human Genetics

Endoplasmic reticulum stress enhances γ-secretase activity

(2011) Kazunori Ohta et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Autophagy Dysregulation in Amyotrophic Lateral Sclerosis

(2011) Sheng Chen et al.   BRAIN PATHOLOGY

Gaucher Disease Glucocerebrosidase and α-Synuclein Form a Bidirectional Pathogenic Loop in Synucleinopathies

(2011) Joseph R. Mazzulli et al.   CELL

Proteomic Signatures in Thapsigargin-Treated Hepatoma Cells

(2011) Giuseppina Amodio et al.   CHEMICAL RESEARCH IN TOXICOLOGY

Protein folding stress in neurodegenerative diseases: a glimpse into the ER

(2011) Soledad Matus et al.   CURRENT OPINION IN CELL BIOLOGY

Protein aggregates in Huntington's disease

(2011) Montserrat Arrasate et al.   EXPERIMENTAL NEUROLOGY

Mutant Atp13a2 proteins involved in parkinsonism are degraded by ER-associated degradation and sensitize cells to ER-stress induced cell death

(2011) Janet Ugolino et al.   HUMAN MOLECULAR GENETICS

The homocysteine-inducible endoplasmic reticulum (ER) stress protein Herp counteracts mutant α-synuclein-induced ER stress via the homeostatic regulation of ER-resident calcium release channel proteins

(2011) Cherine Belal et al.   HUMAN MOLECULAR GENETICS

The ER stress factor XBP1s prevents amyloid-β neurotoxicity

(2011) Sergio Casas-Tinto et al.   HUMAN MOLECULAR GENETICS

Autophagic substrate clearance requires activity of the syntaxin-5 SNARE complex

(2011) M. Renna et al.   JOURNAL OF CELL SCIENCE

Making Connections: Pathology and Genetics Link Amyotrophic Lateral Sclerosis with Frontotemporal Lobe Dementia

(2011) Faisal Fecto et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

(2011) Xi Chen et al.   Molecular Neurodegeneration

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Molecular chaperones in protein folding and proteostasis

(2011) F. Ulrich Hartl et al.   NATURE

Regulation of glucose homeostasis through a XBP-1–FoxO1 interaction

(2011) Yingjiang Zhou et al.   NATURE MEDICINE

Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

(2011) Peter M. Andersen et al.   Nature Reviews Neurology

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

The Unfolded Protein Response: Integrating Stress Signals Through the Stress Sensor IRE1α

(2011) Claudio Hetz et al.   PHYSIOLOGICAL REVIEWS

Dysregulated LRRK2 Signaling in Response to Endoplasmic Reticulum Stress Leads to Dopaminergic Neuron Degeneration in C. elegans

(2011) Yiyuan Yuan et al.   PLoS One

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases-Progress and Prognosis

(2011) S. L. Lindquist et al.   Cold Spring Harbor Perspectives in Biology

Endoplasmic-Reticulum Calcium Depletion and Disease

(2011) D. Mekahli et al.   Cold Spring Harbor Perspectives in Biology

Calcium Signaling in Neuronal Development

(2011) S. S. Rosenberg et al.   Cold Spring Harbor Perspectives in Biology

Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease

(2010) Agnes A. Luty et al.   ANNALS OF NEUROLOGY

Lysosomal Proteolysis and Autophagy Require Presenilin 1 and Are Disrupted by Alzheimer-Related PS1 Mutations

(2010) Ju-Hyun Lee et al.   CELL

Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress

(2010) L Bouman et al.   CELL DEATH AND DIFFERENTIATION

The unfolded protein response in familial amyotrophic lateral sclerosis

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

The Endoplasmic Reticulum Stress Sensor, ATF6α, Protects against Neurotoxin-induced Dopaminergic Neuronal Death

(2010) Naohiro Egawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Induction of the unfolded protein response by α-synuclein in experimental models of Parkinson’s disease

(2010) Arianna Bellucci et al.   JOURNAL OF NEUROCHEMISTRY

Involvement of endoplasmic reticulum stress defined by activated unfolded protein response in multiple system atrophy

(2010) Kouki Makioka et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

α-Synuclein Delays Endoplasmic Reticulum (ER)-to-Golgi Transport in Mammalian Cells by Antagonizing ER/Golgi SNAREs

(2010) Nandhakumar Thayanidhi et al.   MOLECULAR BIOLOGY OF THE CELL

Autophagy gone awry in neurodegenerative diseases

(2010) Esther Wong et al.   NATURE NEUROSCIENCE

Impaired ATF6α processing, decreased Rheb and neuronal cell cycle re-entry in Huntington's disease

(2010) Maria Rosario Fernandez-Fernandez et al.   NEUROBIOLOGY OF DISEASE

Mechanism of ER Stress-Induced Brain Damage by IP3 Receptor

(2010) Takayasu Higo et al.   NEURON

Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

(2010) Hui Yang et al.   PLoS One

Prion Protein Misfolding Affects Calcium Homeostasis and Sensitizes Cells to Endoplasmic Reticulum Stress

(2010) Mauricio Torres et al.   PLoS One

The propagation of prion-like protein inclusions in neurodegenerative diseases

(2010) Michel Goedert et al.   TRENDS IN NEUROSCIENCES

XBP-1 deficiency in the nervous system reveals a homeostatic switch to activate autophagy

(2009) Soledad Matus et al.   Autophagy

Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis

(2009) Adam K. Walker et al.   BRAIN

Preventing oxidative stress: a new role for XBP1

(2009) Y Liu et al.   CELL DEATH AND DIFFERENTIATION

A VAPB mutant linked to amyotrophic lateral sclerosis generates a novel form of organized smooth endoplasmic reticulum

(2009) Elisa Fasana et al.   FASEB JOURNAL

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

(2009) C. Hetz et al.   GENES & DEVELOPMENT

Endoplasmic reticulum stress reduces the export from the ER and alters the architecture of post-ER compartments

(2009) Giuseppina Amodio et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Signalling Pathways in the Unfolded Protein Response: Development from Yeast to Mammals

(2009) K. Mori   JOURNAL OF BIOCHEMISTRY

Caenorhabditits elegansLRK-1 and PINK-1 Act Antagonistically in Stress Response and Neurite Outgrowth

(2009) Julia Sämann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB

(2009) Hiroaki Suzuki et al.   JOURNAL OF NEUROCHEMISTRY

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1

(2008) H. Nishitoh et al.   GENES & DEVELOPMENT

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

(2008) M. L. Duennwald et al.   GENES & DEVELOPMENT

Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis†

(2008) Cristina Cheroni et al.   HUMAN MOLECULAR GENETICS

VAPB interacts with and modulates the activity of ATF6

(2008) C. Gkogkas et al.   HUMAN MOLECULAR GENETICS

Phosphorylation of the Translation Initiation Factor eIF2α Increases BACE1 Levels and Promotes Amyloidogenesis

(2008) Tracy O'Connor et al.   NEURON

Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis

(2008) C. Hetz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE