Dual roles of UPRer and UPRmt in neurodegenerative diseases

c-Jun N-Terminal Kinase Promotes Stress Granule Assembly and Neurodegeneration in C9orf72-Mediated ALS and FTD

(2023) Talanjeri Gopalakrishna Sahana et al.   JOURNAL OF NEUROSCIENCE

The unfolded protein response transcription factor XBP1s ameliorates Alzheimer’s disease by improving synaptic function and proteostasis

(2023) Claudia Duran-Aniotz et al.   MOLECULAR THERAPY

Stress response mechanisms in protein misfolding diseases: Profiling a cellular model of Huntington's disease

(2023) Liliana M. Almeida et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

The Protective Effect of Edaravone on TDP-43 Plus Oxidative Stress-Induced Neurotoxicity in Neuronal Cells: Analysis of Its Neuroprotective Mechanisms Using RNA Sequencing

(2022) Aki Soejima-Kusunoki et al.   Pharmaceuticals

Exome-wide age-of-onset analysis reveals exonic variants in ERN1 and SPPL2C associated with Alzheimer’s disease

(2021) Liang He et al.   Translational Psychiatry

Establishment of a reporter system for monitoring activation of the ER stress transducer ATF6β

(2021) Le Thi Hien et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Single-cell sequencing of human midbrain reveals glial activation and a Parkinson-specific neuronal state

(2021) Semra Smajić et al.   BRAIN

Activation of Mitochondrial Unfolded Protein Response in SHSY5Y Expressing APP Cells and APP/PS1 Mice

(2020) Yang Shen et al.   Frontiers in Cellular Neuroscience

Mechanisms, regulation and functions of the unfolded protein response

(2020) Claudio Hetz et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

Common gene expression signatures in Parkinson’s disease are driven by changes in cell composition

(2020) Gonzalo S. Nido et al.   Acta Neuropathologica Communications

Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer’s disease-like pathology in human cerebral organoids

(2020) María José Pérez et al.   MOLECULAR PSYCHIATRY

Synergy between amyloid-β and tau in Alzheimer’s disease

(2020) Marc Aurel Busche et al.   NATURE NEUROSCIENCE

Folding the Mitochondrial UPR into the Integrated Stress Response

(2020) Nadine S. Anderson et al.   TRENDS IN CELL BIOLOGY

Meta-Analysis of the Alzheimer’s Disease Human Brain Transcriptome and Functional Dissection in Mouse Models

(2020) Ying-Wooi Wan et al.   Cell Reports

Endoplasmic Reticulum Stress and Unfolded Protein Response in Neurodegenerative Diseases

(2020) Rose Ghemrawi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The Mitochondrial Unfolded Protein Response: A Hinge Between Healthy and Pathological Aging

(2020) Francisco Muñoz-Carvajal et al.   Frontiers in Aging Neuroscience

Mitochondrial unfolded protein response: An emerging pathway in human diseases

(2020) Li Zhu et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial UPR repression duringPseudomonas aeruginosainfection requires the bZIP protein ZIP-3

(2019) Pan Deng 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

Mutant huntingtin inhibits the mitochondrial unfolded protein response by impairing ABCB10 mRNA stability

(2019) Zixing Fu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Alpha-synuclein suppresses mitochondrial protease ClpP to trigger mitochondrial oxidative damage and neurotoxicity

(2019) Di Hu et al.   ACTA NEUROPATHOLOGICA

Proteasomal and lysosomal clearance of faulty secretory proteins: ER-associated degradation (ERAD) and ER-to-lysosome-associated degradation (ERLAD) pathways

(2019) Ilaria Fregno et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

(2019) Peng Wang et al.   PLoS Genetics

Atf6α impacts cell number by influencing survival, death and proliferation

(2019) Rohit B. Sharma et al.   Molecular Metabolism

Neurochemical biomarkers in amyotrophic lateral sclerosis

(2019) Federico Verde et al.   CURRENT OPINION IN NEUROLOGY

Parkinson disease

(2019) R. Balestrino et al.   EUROPEAN JOURNAL OF NEUROLOGY

Activation of the Unfolded Protein Response and Proteostasis Disturbance in Parkinsonism-Dementia of Guam

(2019) Bert M Verheijen et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

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

Constitutive XBP-1s-mediated activation of the endoplasmic reticulum unfolded protein response protects against pathological tau

(2019) Sarah M. Waldherr et al.   Nature Communications

Ginseng protein protects against mitochondrial dysfunction and neurodegeneration by inducing mitochondrial unfolded protein response in Drosophila melanogaster PINK1 model of Parkinson's disease

(2019) Meichen Liu et al.   JOURNAL OF ETHNOPHARMACOLOGY

Knockdown of GADD34 in neonatal mutant SOD1 mice ameliorates ALS

(2019) Ghanashyam D. Ghadge et al.   NEUROBIOLOGY OF DISEASE

UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication

(2018) Andrew Melber et al.   CELL RESEARCH

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

C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation

(2018) Weiwei Cheng et al.   Nature Communications

The different axes of the mammalian mitochondrial unfolded protein response

(2018) Christian Münch   BMC BIOLOGY

Tauopathy-Associated PERK Alleles are Functional Hypomorphs that Increase Neuronal Vulnerability to ER Stress

(2018) Shauna H Yuan et al.   HUMAN MOLECULAR GENETICS

FUS interacts with ATP synthase beta subunit and induces mitochondrial unfolded protein response in cellular and animal models

(2018) Jianwen Deng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Early Events in the Endoplasmic Reticulum Unfolded Protein Response

(2018) Steffen Preissler et al.   Cold Spring Harbor Perspectives in Biology

Huntington's disease: a clinical review

(2017) P. McColgan et al.   EUROPEAN JOURNAL OF NEUROLOGY

The endoplasmic reticulum: A hub of protein quality control in health and disease

(2017) Lisa Vincenz-Donnelly et al.   FREE RADICAL BIOLOGY AND MEDICINE

Sex specific activation of the ERα axis of the mitochondrial UPR (UPRmt) in the G93A-SOD1 mouse model of familial ALS

(2017) Amanjot K Riar et al.   HUMAN MOLECULAR GENETICS

Alpha-synuclein induces the unfolded protein response in Parkinson’s disease SNCA triplication iPSC-derived neurons

(2017) Sabrina M Heman-Ackah et al.   HUMAN MOLECULAR GENETICS

Dysregulation of the Mitochondrial Unfolded Protein Response Induces Non-Apoptotic Dopaminergic Neurodegeneration inC. elegansModels of Parkinson's Disease

(2017) Bryan A. Martinez et al.   JOURNAL OF NEUROSCIENCE

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

Enhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity

(2017) Vincenzo Sorrentino et al.   NATURE

Regulating Secretory Proteostasis through the Unfolded Protein Response: From Function to Therapy

(2017) Lars Plate et al.   TRENDS IN CELL BIOLOGY

Pathology of Neurodegenerative Diseases

(2017) Brittany N. Dugger et al.   Cold Spring Harbor Perspectives in Biology

Activation of the mitochondrial unfolded protein response promotes longevity and dopamine neuron survival in Parkinson’s disease models

(2017) Jason F. Cooper et al.   Scientific Reports

Evidence for Mitochondrial UPR Gene Activation in Familial and Sporadic Alzheimer's Disease

(2016) John S. Beck et al.   Current Alzheimer Research

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

The transcription factor XBP1s restores hippocampal synaptic plasticity and memory by control of the Kalirin-7 pathway in Alzheimer model

(2016) M Cissé et al.   MOLECULAR PSYCHIATRY

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

(2016) Sebum Lee et al.   NEURON

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

(2015) Helois Radford et al.   ACTA NEUROPATHOLOGICA

A conformational RNA zipper promotes intron ejection during non-conventional XBP1 mRNA splicing

(2015) J. Peschek et al.   EMBO REPORTS

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

(2015) Huikyong Lee et al.   MOLECULAR NEUROBIOLOGY

Proteostasis control by the unfolded protein response

(2015) Claudio Hetz et al.   NATURE CELL BIOLOGY

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

(2015) Mercedes Prudencio et al.   NATURE NEUROSCIENCE

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

LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins

(2015) Ayenachew Bezawork-Geleta et al.   Scientific Reports

Axonally Synthesized ATF4 Transmits a Neurodegenerative Signal across Brain Regions

(2014) Jimena Baleriola et al.   CELL

The Protein Import Machinery of Mitochondria—A Regulatory Hub in Metabolism, Stress, and Disease

(2014) Angelika B. Harbauer et al.   Cell Metabolism

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

Unfolded protein response signaling by transcription factor XBP-1 regulates ADAM10 and is affected in Alzheimer's disease

(2013) Sven Reinhardt et al.   FASEB JOURNAL

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

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

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

(2013) Soledad Matus et al.   PLoS One

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

(2013) Julia Leitman et al.   Nature Communications

AAV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington’s disease

(2012) Amparo Zuleta et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis

(2012) T. Prell et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

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

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