The unfolded protein response in neurodegenerative diseases: a neuropathological perspective

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

(2015) I. Das et al.   SCIENCE

Mutations in a translation initiation factor identify the target of a memory-enhancing compound

(2015) Y. Sekine et al.   SCIENCE

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

(2015) Carmela Sidrauski et al.   eLife

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

(2014) Yong-Jie Zhang et al.   ACTA NEUROPATHOLOGICA

Neuropathology and biochemistry of Aβ and its aggregates in Alzheimer’s disease

(2014) Dietmar Rudolf Thal et al.   ACTA NEUROPATHOLOGICA

Hepatitis C virus core protein activates autophagy through EIF2AK3 and ATF6 UPR pathway-mediated MAP1LC3B and ATG12 expression

(2014) Ji Wang et al.   Autophagy

Allosteric Inhibition of the IRE1α RNase Preserves Cell Viability and Function during Endoplasmic Reticulum Stress

(2014) Rajarshi Ghosh et al.   CELL

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

ER-associated degradation: Protein quality control and beyond

(2014) Annamaria Ruggiano et al.   JOURNAL OF CELL BIOLOGY

Druggable sensors of the unfolded protein response

(2014) Dustin J Maly et al.   Nature Chemical Biology

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

(2014) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

Granulovacuolar degeneration and unfolded protein response in mouse models of tauopathy and Aβ amyloidosis

(2014) Christoph Köhler et al.   NEUROBIOLOGY OF DISEASE

The unfolded protein response mediates reversible tau phosphorylation induced by metabolic stress

(2014) J M van der Harg et al.   Cell Death & Disease

Structure and mechanism of action of the hydroxy–aryl–aldehyde class of IRE1 endoribonuclease inhibitors

(2014) Mario Sanches et al.   Nature Communications

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

(2014) Brian J. Wainger et al.   Cell Reports

Modeling Alzheimer’s Disease with iPSCs Reveals Stress Phenotypes Associated with Intracellular Aβ and Differential Drug Responsiveness

(2013) Takayuki Kondo et al.   Cell Stem Cell

The eIF2α kinases: their structures and functions

(2013) Neysan Donnelly et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Tau Pathology as a Cause and Consequence of the UPR

(2013) B. J. Stoveken   JOURNAL OF NEUROSCIENCE

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

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

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

(2013) Tao Ma et al.   NATURE NEUROSCIENCE

Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo

(2013) Alexandra Vaccaro et al.   NEUROBIOLOGY OF DISEASE

Identification and Rescue of  -Synuclein Toxicity in Parkinson Patient-Derived Neurons

(2013) C. Y. Chung et al.   SCIENCE

A New PERKspective on Neurodegeneration

(2013) W. Scheper et al.   Science Translational Medicine

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

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

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

Discovery of 7-Methyl-5-(1-{[3-(trifluoromethyl)phenyl]acetyl}-2,3-dihydro-1H-indol-5-yl)-7H-pyrrolo[2,3-d]pyrimidin-4-amine (GSK2606414), a Potent and Selective First-in-Class Inhibitor of Protein Kinase R (PKR)-like Endoplasmic Reticulum Kinase (PERK)

(2012) Jeffrey M. Axten et al.   JOURNAL OF MEDICINAL CHEMISTRY

Endoplasmic reticulum dysfunction in neurological disease

(2012) Benoit D Roussel et al.   LANCET NEUROLOGY

Divergent allosteric control of the IRE1α endoribonuclease using kinase inhibitors

(2012) Likun Wang et al.   Nature Chemical Biology

An exploratory study on STX6, MOBP, MAPT, and EIF2AK3 and late-onset Alzheimer's disease

(2012) Qiu-Yan Liu et al.   NEUROBIOLOGY OF AGING

The molecular basis for selective inhibition of unconventional mRNA splicing by an IRE1-binding small molecule

(2012) B. C. S. Cross et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stages of granulovacuolar degeneration: their relation to Alzheimer’s disease and chronic stress response

(2011) Dietmar Rudolf Thal et al.   ACTA NEUROPATHOLOGICA

The unfolded protein response and proteostasis in Alzheimer disease

(2011) Wiep Scheper et al.   Autophagy

Endoplasmic reticulum stress activates autophagy but not the proteasome in neuronal cells: implications for Alzheimer's disease

(2011) D A T Nijholt et al.   CELL DEATH AND DIFFERENTIATION

IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux

(2011) Huikyong Lee et al.   HUMAN MOLECULAR GENETICS

A functional haplotype inEIF2AK3, an ER stress sensor, is associated with lower bone mineral density

(2011) Jie Liu et al.   JOURNAL OF BONE AND MINERAL RESEARCH

The unfolded protein response is associated with early tau pathology in the hippocampus of tauopathies

(2011) Diana AT Nijholt et al.   JOURNAL OF PATHOLOGY

Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy

(2011) Günter U Höglinger et al.   NATURE GENETICS

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

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

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

(2011) P. Tsaytler et al.   SCIENCE

Regulation of basal cellular physiology by the homeostatic unfolded protein response

(2010) D. Thomas Rutkowski et al.   JOURNAL OF CELL BIOLOGY

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

(2010) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

Induction of Liver Steatosis and Lipid Droplet Formation in ATF6α-Knockout Mice Burdened with Pharmacological Endoplasmic Reticulum Stress

(2010) Keisuke Yamamoto et al.   MOLECULAR BIOLOGY OF THE CELL

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

The Unfolded Protein Response Is Activated in Pretangle Neurons in Alzheimer's Disease Hippocampus

(2009) Jeroen J.M. Hoozemans et al.   AMERICAN JOURNAL OF PATHOLOGY

IRE1α Kinase Activation Modes Control Alternate Endoribonuclease Outputs to Determine Divergent Cell Fates

(2009) Dan Han et al.   CELL

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

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

Rrs1 Is Involved in Endoplasmic Reticulum Stress Response in Huntington Disease

(2009) Alisia Carnemolla et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fine-Tuning of the Unfolded Protein Response: Assembling the IRE1α Interactome

(2009) Claudio Hetz et al.   MOLECULAR CELL

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

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

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

Genetic Variation of Promoter Sequence Modulates XBP1 Expression and Genetic Risk for Vitiligo

(2009) Yunqing Ren et al.   PLoS Genetics

Oligomer-specific Aβ toxicity in cell models is mediated by selective uptake

(2008) Sidhartha M. Chafekar et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The Unfolded Protein Response: A Pathway That Links Insulin Demand with β-Cell Failure and Diabetes

(2008) Donalyn Scheuner et al.   ENDOCRINE REVIEWS

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