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

eIF2B activator prevents neurological defects caused by a chronic integrated stress response

(2019) Yao Liang Wong et al.   eLife

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

An Intramolecular Salt Bridge Linking TDP43 RNA Binding, Protein Stability, and TDP43-Dependent Neurodegeneration

(2019) Brittany N. Flores et al.   Cell Reports

The Unfolded Protein Response and Cell Fate Control

(2018) Claudio Hetz et al.   MOLECULAR CELL

CRISPR–Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity

(2018) Nicholas J. Kramer et al.   NATURE GENETICS

Binding of ISRIB reveals a regulatory site in the nucleotide exchange factor eIF2B

(2018) Alisa F. Zyryanova et al.   SCIENCE

Structure of the nucleotide exchange factor eIF2B reveals mechanism of memory-enhancing molecule

(2018) Jordan C. Tsai et al.   SCIENCE

Noncanonical Modulation of the eIF2 Pathway Controls an Increase in Local Translation during Neural Wiring

(2018) Roberta Cagnetta et al.   MOLECULAR CELL

ER stress and the unfolded protein response in neurodegeneration

(2017) Claudio Hetz et al.   Nature Reviews Neurology

E46K α-synuclein pathological mutation causes cell-autonomous toxicity without altering protein turnover or aggregation

(2017) Ignacio Íñigo-Marco et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2017) Ana Crespillo-Casado et al.   eLife

Amyotrophic lateral sclerosis

(2017) Orla Hardiman et al.   Nature Reviews Disease Primers

The integrated stress response

(2016) Karolina Pakos‐Zebrucka et al.   EMBO REPORTS

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Nrf2 mitigates LRRK2- and α-synuclein–induced neurodegeneration by modulating proteostasis

(2016) Gaia Skibinski et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATF4-dependent induction of heme oxygenase 1 prevents anoikis and promotes metastasis

(2015) Souvik Dey et al.   JOURNAL OF CLINICAL INVESTIGATION

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

(2015) Mercedes Prudencio et al.   NATURE NEUROSCIENCE

Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS

(2015) Shuying Sun et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

(2015) I. Das et al.   SCIENCE

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

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

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

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

The mammalian tRNA ligase complex mediates splicing ofXBP1mRNA and controls antibody secretion in plasma cells

(2014) Jennifer Jurkin et al.   EMBO JOURNAL

A Synthetic Biology Approach Identifies the Mammalian UPR RNA Ligase RtcB

(2014) Yanyan Lu et al.   MOLECULAR CELL

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

(2014) Sami J Barmada 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

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

(2014) Artem Kaplan et al.   NEURON

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

Getting RIDD of RNA: IRE1 in cell fate regulation

(2014) M. Maurel et al.   TRENDS IN BIOCHEMICAL SCIENCES

An enhanced integrated stress response ameliorates mutant SOD1-induced ALS

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

ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

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

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

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

(2013) Alexandra Vaccaro et al.   NEUROBIOLOGY OF DISEASE

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

(2013) Soledad Matus et al.   PLoS One

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

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

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

Identifying polyglutamine protein species in situ that best predict neurodegeneration

(2011) Jason Miller et al.   Nature Chemical Biology

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

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

The unfolded protein response in familial amyotrophic lateral sclerosis

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

Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

In situ visualization and dynamics of newly synthesized proteins in rat hippocampal neurons

(2010) Daniela C Dieterich et al.   NATURE NEUROSCIENCE

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

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

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

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

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

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

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

(2008) Nicola J. Rutherford et al.   PLoS Genetics