Endoplasmic Reticulum Stress and Unfolded Protein Response in Neurodegenerative Diseases

Activating transcription factor 6 reduces Aβ1–42 and restores memory in Alzheimer’s disease model mice

(2020) Yayun Du et al.   INTERNATIONAL JOURNAL OF NEUROSCIENCE

Sephin1 Reduces Prion Infection in Prion-Infected Cells and Animal Model

(2020) Simrika Thapa et al.   MOLECULAR NEUROBIOLOGY

Astrocyte Unfolded Protein Response Induces a Specific Reactivity State that Causes Non-Cell-Autonomous Neuronal Degeneration

(2020) Heather L. Smith et al.   NEURON

Unfolding the mystery of UPR in astrocytes

(2020) Gilbert Gallardo   Science Translational Medicine

The Emerging Roles of Endoplasmic Reticulum Stress in Balancing Immunity and Tolerance in Health and Diseases: Mechanisms and Opportunities

(2020) Anqi Li et al.   Frontiers in Immunology

The PERK-Dependent Molecular Mechanisms as a Novel Therapeutic Target for Neurodegenerative Diseases

(2020) Wioletta Rozpędek-Kamińska et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Molecular Mechanisms of ER Stress and UPR in the Pathogenesis of Alzheimer’s Disease

(2020) Md. Sahab Uddin et al.   MOLECULAR NEUROBIOLOGY

IRE1 signaling pathway mediates protective autophagic response against manganese-induced neuronal apoptosis in vivo and in vitro

(2020) Chang Liu et al.   SCIENCE OF THE TOTAL ENVIRONMENT

Fine tuning of the unfolded protein response by ISRIB improves neuronal survival in a model of amyotrophic lateral sclerosis

(2020) Ricardo Bugallo et al.   Cell Death & Disease

Autophagy lipidation machinery regulates axonal microtubule dynamics but is dispensable for survival of mammalian neurons

(2020) A. Negrete-Hurtado et al.   Nature Communications

Roles of VMP1 in Autophagy and ER–Membrane Contact: Potential Implications in Neurodegenerative Disorders

(2020) Panpan Wang et al.   Frontiers in Molecular Neuroscience

From basic research to the clinic: innovative therapies for ALS and FTD in the pipeline

(2020) Rajka Maria Liscic et al.   Molecular Neurodegeneration

The C/EBP Homologous Protein (CHOP) Transcription Factor Functions in Endoplasmic Reticulum Stress-Induced Apoptosis and Microbial Infection

(2019) Hai Hu et al.   Frontiers in Immunology

The Role of NMDA Receptors in Alzheimer’s Disease

(2019) Jinping Liu et al.   Frontiers in Neuroscience

Reaction to Endoplasmic Reticulum Stress via ATF6 in Amyotrophic Lateral Sclerosis Deteriorates With Aging

(2019) Tino Prell et al.   Frontiers in Aging Neuroscience

Small molecule ISRIB suppresses the integrated stress response within a defined window of activation

(2019) Huib H. Rabouw et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS

(2019) Yulia Dzhashiashvili et al.   NEUROBIOLOGY OF DISEASE

ATG5 and ATG7 induced autophagy interplays with UPR via PERK signaling

(2019) Wei Zheng et al.   Cell Communication and Signaling

The unfolded protein response modulators GSK2606414 and KIRA6 are potent KIT inhibitors

(2019) Mohamed Mahameed et al.   Cell Death & Disease

Inhibition of eIF2α dephosphorylation accelerates pterostilbene-induced cell death in human hepatocellular carcinoma cells in an ER stress and autophagy-dependent manner

(2019) Chen-Lin Yu et al.   Cell Death & Disease

Linking the Endoplasmic Reticulum to Parkinson’s Disease and Alpha-Synucleinopathy

(2019) Emanuela Colla   Frontiers in Neuroscience

Prion disease is accelerated in mice lacking stress-induced heat shock protein 70 (HSP70)

(2019) Charles E. Mays et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Effect of Kinase Inhibiting RNase Attenuator (KIRA) Compounds on the Formation of Face-to-Face Dimers of Inositol-Requiring Enzyme 1: Insights from Computational Modeling

(2019) Carlesso et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Manganese activates autophagy to alleviate endoplasmic reticulum stress–induced apoptosis via PERK pathway

(2019) Chang Liu et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Dysregulation of AMPA receptor subunit expression in sporadic ALS post‐mortem brain

(2019) Jenna M Gregory et al.   JOURNAL OF PATHOLOGY

IRE1 promotes neurodegeneration through autophagy-dependent neuron death in the Drosophila model of Parkinson’s disease

(2019) Cheng Yan et al.   Cell Death & Disease

Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease

(2019) Eric M. Clark et al.   Disease Models & Mechanisms

Cell-based HTS identifies a chemical chaperone for preventing ER protein aggregation and proteotoxicity

(2019) Keisuke Kitakaze et al.   eLife

Alpha‐synuclein aggregates activate calcium pump SERCA leading to calcium dysregulation

(2018) Cristine Betzer et al.   EMBO REPORTS

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

(2018) Daniel Hughes et al.   FEBS Journal

PERK inhibition delays neurodegeneration and improves motor function in a mouse model of Marinesco-Sjögren syndrome

(2018) Valentina Grande et al.   HUMAN MOLECULAR GENETICS

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

(2018) Gabriela Mercado et al.   NEUROBIOLOGY OF DISEASE

Crosstalk between endoplasmic reticulum stress and brain inflammation in Alzheimer's disease

(2018) Luis E. Santos et al.   NEUROPHARMACOLOGY

Autophagy at the synapse

(2018) Veronica Birdsall et al.   NEUROSCIENCE LETTERS

ER–mitochondria signaling in Parkinson’s disease

(2018) Patricia Gómez-Suaga et al.   Cell Death & Disease

The role of the mitochondria and the endoplasmic reticulum contact sites in the development of the immune responses

(2018) Denis Martinvalet   Cell Death & Disease

A key role for MAM in mediating mitochondrial dysfunction in Alzheimer disease

(2018) Estela Area-Gomez et al.   Cell Death & Disease

C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity

(2018) Bhuvaneish T. Selvaraj et al.   Nature Communications

Association Between Autophagy and Neurodegenerative Diseases

(2018) Nobuhiro Fujikake et al.   Frontiers in Neuroscience

Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins

(2018) Ryan Paxman et al.   eLife

Sephin1, which prolongs the integrated stress response, is a promising therapeutic for multiple sclerosis

(2018) Yanan Chen et al.   BRAIN

ER stress is not elevated in the 5XFAD mouse model of Alzheimer's disease

(2018) Katherine R. Sadleir et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Interplay Between the Unfolded Protein Response and Immune Function in the Development of Neurodegenerative Diseases

(2018) Paulina García-González et al.   Frontiers in Immunology

Emerging roles of ER stress in the etiology and pathogenesis of Alzheimer's disease

(2017) Yannis Gerakis et al.   FEBS Journal

Local Inhibition of PERK Enhances Memory and Reverses Age-Related Deterioration of Cognitive and Neuronal Properties

(2017) Vijendra Sharma et al.   JOURNAL OF NEUROSCIENCE

ER stress and the unfolded protein response in neurodegeneration

(2017) Claudio Hetz et al.   Nature Reviews Neurology

PKR involvement in Alzheimer’s disease

(2017) Jacques Hugon et al.   Alzheimers Research & Therapy

Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS

(2017) Claire E. Hall et al.   Cell Reports

PPP1R15A-mediated dephosphorylation of eIF2α is unaffected by Sephin1 or Guanabenz

(2017) Ana Crespillo-Casado et al.   eLife

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

(2017) Paolo Remondelli et al.   Frontiers in Molecular Neuroscience

Protein misfolding in neurodegenerative diseases: implications and strategies

(2017) Patrick Sweeney et al.   Translational Neurodegeneration

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

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

XBP1-FoxO1 interaction regulates ER stress-induced autophagy in auditory cells

(2017) Akihiro Kishino et al.   Scientific Reports

ALS and FTD: an epigenetic perspective

(2016) Veronique V. Belzil et al.   ACTA NEUROPATHOLOGICA

The role of endoplasmic reticulum stress in neurodegenerative disease

(2016) Chunchen Xiang et al.   APOPTOSIS

Interplay of endoplasmic reticulum stress and autophagy in neurodegenerative disorders

(2016) Yu Cai et al.   Autophagy

The stress of prion disease

(2016) Charles E. Mays et al.   BRAIN RESEARCH

ALS‐linked protein disulfide isomerase variants cause motor dysfunction

(2016) Ute Woehlbier et al.   EMBO JOURNAL

The Transcription Factor XBP1 in Memory and Cognition: Implications in Alzheimer’s Disease

(2016) Moustapha Cissé et al.   MOLECULAR MEDICINE

Regulation of SET Gene Expression by NFkB

(2016) Yi Feng et al.   MOLECULAR NEUROBIOLOGY

Plasma tau in Alzheimer disease

(2016) Niklas Mattsson et al.   NEUROLOGY

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

(2016) Sebum Lee et al.   NEURON

α-Synuclein–induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models

(2016) Joseph R. Mazzulli et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Epidemiology of Parkinson's disease

(2016) A. Elbaz et al.   REVUE NEUROLOGIQUE

Regulation of Memory Formation by the Transcription Factor XBP1

(2016) Gabriela Martínez et al.   Cell Reports

Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation

(2016) Lars Plate et al.   eLife

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

(2015) Helois Radford et al.   ACTA NEUROPATHOLOGICA

Calcium signaling at ER membrane contact sites

(2015) Thomas Burgoyne et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The endoplasmic reticulum: structure, function and response to cellular signaling

(2015) Dianne S. Schwarz et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients

(2015) Paloma Gonzalez-Perez et al.   GENE

α-Synuclein-mediated inhibition of ATF6 processing into COPII vesicles disrupts UPR signaling in Parkinson's disease

(2015) Joel J. Credle et al.   NEUROBIOLOGY OF DISEASE

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

Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice

(2015) Dapeng Chen et al.   Frontiers in Cellular Neuroscience

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

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

Specific Downregulation of Hippocampal ATF4 Reveals a Necessary Role in Synaptic Plasticity and Memory

(2015) Silvia Pasini et al.   Cell Reports

Noncanonical binding of BiP ATPase domain to Ire1 and Perk is dissociated by unfolded protein CH1 to initiate ER stress signaling

(2015) Marta Carrara et al.   eLife

The small molecule ISRIB reverses the effects of eIF2α phosphorylation on translation and stress granule assembly

(2015) Carmela Sidrauski et al.   eLife

Crosstalk between the Unfolded Protein Response and NF-κB-Mediated Inflammation in the Progression of Chronic Kidney Disease

(2015) Zahraa Mohammed-Ali et al.   Journal of Immunology Research

New insights into the roles of CHOP-induced apoptosis in ER stress

(2014) Yiming Li et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Alpha-synuclein in peripheral tissues and body fluids as a biomarker for Parkinson's disease - a systematic review

(2014) N. Malek et al.   ACTA NEUROLOGICA SCANDINAVICA

Salubrinal protects against rotenone-induced SH-SY5Y cell death via ATF4-parkin pathway

(2014) Liang Wu et al.   BRAIN RESEARCH

GRP78 clustering at the cell surface of neurons transduces the action of exogenous alpha-synuclein

(2014) S Bellani et al.   CELL DEATH AND DIFFERENTIATION

Protein Misfolding and Aggregation in Alzheimer’s Disease and Type 2 Diabetes Mellitus

(2014) Ghulam Ashraf et al.   CNS & Neurological Disorders-Drug Targets

Serum Calreticulin Is a Negative Biomarker in Patients with Alzheimer’s Disease

(2014) Qiao Lin et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Apoptosis Signal Regulating Kinase 1 (ASK1): Potential as a Therapeutic Target for Alzheimer’s Disease

(2014) Juhyun Song et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

PERK-Dependent Activation of JAK1 and STAT3 Contributes to Endoplasmic Reticulum Stress-Induced Inflammation

(2014) G. P. Meares et al.   MOLECULAR AND CELLULAR BIOLOGY

PERK mediates eIF2α phosphorylation responsible for BACE1 elevation, CREB dysfunction and neurodegeneration in a mouse model of Alzheimer's disease

(2014) Latha Devi et al.   NEUROBIOLOGY OF AGING

Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis

(2014) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

Guanabenz delays the onset of disease symptoms, extends lifespan, improves motor performance and attenuates motor neuron loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis

(2014) H.-Q. Jiang et al.   NEUROSCIENCE

Control of dopaminergic neuron survival by the unfolded protein response transcription factor XBP1

(2014) P. Valdes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An enhanced integrated stress response ameliorates mutant SOD1-induced ALS

(2013) L. Wang et al.   HUMAN MOLECULAR GENETICS

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

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

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models

(2013) Hyung-Jun Kim et al.   NATURE GENETICS

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

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

Endoplasmic Reticulum Structure and Interconnections with Other Organelles

(2013) A. R. English et al.   Cold Spring Harbor Perspectives in Biology

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

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

(2013) Lauren D Stutzbach et al.   Acta Neuropathologica Communications

Endoplasmic reticulum stress and the ER mitochondria calcium cycle in amyotrophic lateral sclerosis

(2012) Janin Lautenschlaeger et al.   Amyotrophic Lateral Sclerosis

Transmissible Proteins: Expanding the Prion Heresy

(2012) Claudio Soto   CELL

Neuronal autophagy and neurodegenerative diseases

(2012) Jin H. Son et al.   EXPERIMENTAL AND MOLECULAR MEDICINE

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

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

TUDCA, a Bile Acid, Attenuates Amyloid Precursor Protein Processing and Amyloid-β Deposition in APP/PS1 Mice

(2012) Ana F. Nunes et al.   MOLECULAR NEUROBIOLOGY

Molecular Chaperones, Alpha-Synuclein, and Neurodegeneration

(2012) Stephan N. Witt   MOLECULAR NEUROBIOLOGY

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

100 years of Lewy pathology

(2012) Michel Goedert et al.   Nature Reviews Neurology

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

An IFN- -stimulated ATF6-C/EBP- -signaling pathway critical for the expression of Death Associated Protein Kinase 1 and induction of autophagy

(2012) P. Gade et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Osh Proteins Regulate Phosphoinositide Metabolism at ER-Plasma Membrane Contact Sites

(2011) Christopher J. Stefan et al.   CELL

Autophagy: Renovation of Cells and Tissues

(2011) Noboru Mizushima et al.   CELL

The ER stress factor XBP1s prevents amyloid-β neurotoxicity

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

Proteasomal Dysfunction and Endoplasmic Reticulum Stress Enhance Trafficking of Prion Protein Aggregates through the Secretory Pathway and Increase Accumulation of Pathologic Prion Protein

(2011) Max Nunziante et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Proteolytic processing of Alzheimer’s β-amyloid precursor protein

(2011) Han Zhang et al.   JOURNAL OF NEUROCHEMISTRY

What Genetics Tells us About the Causes and Mechanisms of Parkinson's Disease

(2011) Olga Corti et al.   PHYSIOLOGICAL REVIEWS

ER Tubules Mark Sites of Mitochondrial Division

(2011) J. R. Friedman et al.   SCIENCE

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis

(2011) P. Tsaytler et al.   SCIENCE

The unfolded protein response in familial amyotrophic lateral sclerosis

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

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

(2010) Arianna Bellucci et al.   JOURNAL OF NEUROCHEMISTRY

Endoplasmic Reticulum Stress in Motor Neurons of the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis

(2010) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Autophagy gone awry in neurodegenerative diseases

(2010) Esther Wong et al.   NATURE NEUROSCIENCE

Parkinson’s disease in China

(2010) You-yong Tian et al.   NEUROLOGICAL SCIENCES

Activation of PERK Signaling Attenuates Aβ-Mediated ER Stress

(2010) Do Yeon Lee et al.   PLoS One

ASK1 Overexpression Accelerates Paraquat-Induced Autophagy via Endoplasmic Reticulum Stress

(2010) Mireia Niso-Santano et al.   TOXICOLOGICAL SCIENCES

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

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

Regulated Ire1-dependent decay of messenger RNAs in mammalian cells

(2009) Julie Hollien et al.   JOURNAL OF CELL BIOLOGY

The role of tau in neurodegeneration

(2009) Tania F Gendron et al.   Molecular Neurodegeneration

Genetics of motor neuron disorders: new insights into pathogenic mechanisms

(2009) Patrick A. Dion et al.   NATURE REVIEWS GENETICS

Expression of inflammatory genes induced by beta-amyloid peptides in human brain endothelial cells and in Alzheimer's brain is mediated by the JNK-AP1 signaling pathway

(2009) Vanja Vukic et al.   NEUROBIOLOGY OF DISEASE

Involvement of CHOP, an ER-stress apoptotic mediator, in both human sporadic ALS and ALS model mice

(2009) Yasushi Ito et al.   NEUROBIOLOGY OF DISEASE

Prion protein biosynthesis and its emerging role in neurodegeneration

(2009) Oishee Chakrabarti et al.   TRENDS IN BIOCHEMICAL SCIENCES

How IRE1 Reacts to ER Stress

(2008) David Ron et al.   CELL

Reduced Translocation of Nascent Prion Protein During ER Stress Contributes to Neurodegeneration

(2008) Neena S. Rane et al.   DEVELOPMENTAL CELL

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

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

VAPB interacts with and modulates the activity of ATF6

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

Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

(2008) Julie D. Atkin et al.   NEUROBIOLOGY OF DISEASE

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