The Unfolded Protein Responses in Health, Aging, and Neurodegeneration: Recent Advances and Future Considerations

Enforced dimerization between XBP1s and ATF6f enhances the protective effects of the UPR in models of neurodegeneration

(2021) René L. Vidal et al.   MOLECULAR THERAPY

Differential roles of GDF15 and FGF21 in systemic metabolic adaptation to the mitochondrial integrated stress response

(2021) Seul Gi Kang et al.   iScience

Multi-OMICS study of a CHCHD10 variant causing ALS demonstrates metabolic rewiring and activation of endoplasmic reticulum and mitochondrial unfolded protein responses

(2021) Isabella R Straub et al.   HUMAN MOLECULAR GENETICS

Parkinson's disease

(2021) Bastiaan R Bloem et al.   LANCET

Cell-type-specific disruption of PERK-eIF2α signaling in dopaminergic neurons alters motor and cognitive function

(2021) Francesco Longo et al.   MOLECULAR PSYCHIATRY

Mutagenesis screen uncovers lifespan extension through integrated stress response inhibition without reduced mRNA translation

(2021) Maxime J. Derisbourg et al.   Nature Communications

Alzheimer disease

(2021) David S. Knopman et al.   Nature Reviews Disease Primers

UPRmt activation protects against MPP+-induced toxicity in a cell culture model of Parkinson's disease

(2021) Di Hu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Redox metabolism: ROS as specific molecular regulators of cell signaling and function

(2021) Claudia Lennicke et al.   MOLECULAR CELL

The memory of neuronal mitochondrial stress is inherited transgenerationally via elevated mitochondrial DNA levels

(2021) Qian Zhang et al.   NATURE CELL BIOLOGY

The mitochondrial permeability transition pore activates the mitochondrial unfolded protein response and promotes aging

(2021) Suzanne Angeli et al.   eLife

Common features of aging fail to occur in Drosophila raised without a bacterial microbiome

(2021) Arvind Kumar Shukla et al.   iScience

Pharmacological targeting of endoplasmic reticulum stress in disease

(2021) Stefan J. Marciniak et al.   NATURE REVIEWS DRUG DISCOVERY

A Conserved Mito-Cytosolic Translational Balance Links Two Longevity Pathways

(2020) Marte Molenaars et al.   Cell Metabolism

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

Two conserved epigenetic regulators prevent healthy ageing

(2020) Jie Yuan et al.   NATURE

Four glial cells regulate ER stress resistance and longevity via neuropeptide signaling in C. elegans

(2020) Ashley E. Frakes et al.   SCIENCE

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

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

The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

(2020) Yevgeniya A. Abramzon et al.   Frontiers in Neuroscience

UPRER promotes lipophagy independent of chaperones to extend life span

(2020) Joseph R. Daniele et al.   Science Advances

Tissue‐selective regulation of protein homeostasis and unfolded protein response signalling in sporadic ALS

(2020) Luigi Montibeller et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

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

Amyotrophic lateral sclerosis: a clinical review

(2020) P. Masrori et al.   EUROPEAN JOURNAL OF NEUROLOGY

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

Pharmacologic IRE1/XBP1s activation confers targeted ER proteostasis reprogramming

(2020) Julia M. D. Grandjean et al.   Nature Chemical Biology

Overexpression of PGC-1α influences the mitochondrial unfolded protein response (mtUPR) induced by MPP+ in human SH-SY5Y neuroblastoma cells

(2020) Yousheng Cai et al.   Scientific Reports

NuRD mediates mitochondrial stress–induced longevity via chromatin remodeling in response to acetyl-CoA level

(2020) Di Zhu et al.   Science Advances

Beyond the cell factory: Homeostatic regulation of and by the UPRER

(2020) Melissa G. Metcalf et al.   Science Advances

Microbial Stimulation Reverses the Age-Related Decline in M Cells in Aged Mice

(2020) David S. Donaldson et al.   iScience

Unfolded protein response activation in C9orf72 frontotemporal dementia is associated with dipeptide pathology and granulovacuolar degeneration in granule cells

(2020) Priya Gami‐Patel et al.   BRAIN PATHOLOGY

4E-BP1 protects neurons from misfolded protein stress and Parkinson’s disease toxicity by inducing the mitochondrial unfolded protein response

(2020) Somasish Ghosh Dastidar et al.   JOURNAL OF NEUROSCIENCE

Histone deacetylase HDA-1 modulates mitochondrial stress response and longevity

(2020) Li-Wa Shao et al.   Nature Communications

Atf-6 Regulates Lifespan through ER-Mitochondrial Calcium Homeostasis

(2020) Kristopher Burkewitz et al.   Cell Reports

HSB-1/HSF-1 pathway modulates histone H4 in mitochondria to control mtDNA transcription and longevity

(2020) Surojit Sural et al.   Science Advances

Tyramine Acts Downstream of Neuronal XBP-1s to Coordinate Inter-tissue UPRER Activation and Behavior in C. elegans

(2020) Neşem P. Özbey et al.   DEVELOPMENTAL CELL

Distinct temporal actions of different types of unfolded protein responses during aging

(2020) Yi Sheng et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Cellular proteostasis decline in human senescence

(2020) Niv Sabath et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Divergent Nodes of Non-autonomous UPRER Signaling through Serotonergic and Dopaminergic Neurons

(2020) Ryo Higuchi-Sanabria et al.   Cell Reports

Mitochondrial dysfunction induces RNA interference in C. elegans through a pathway homologous to the mammalian RIG-I antiviral response

(2020) Kai Mao et al.   PLOS BIOLOGY

Small molecule cognitive enhancer reverses age-related memory decline in mice

(2020) Karen Krukowski et al.   eLife

Cell non-autonomous regulation of health and longevity

(2020) Hillary A Miller et al.   eLife

Health benefits attributed to 17α-estradiol, a lifespan-extending compound, are mediated through estrogen receptor α

(2020) Shivani N Mann et al.   eLife

SUMO peptidase ULP-4 regulates mitochondrial UPR-mediated innate immunity and lifespan extension

(2019) Kaiyu Gao et al.   eLife

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

(2019) Yulia Dzhashiashvili et al.   NEUROBIOLOGY OF DISEASE

The Genetics of Aging: A Vertebrate Perspective

(2019) Param Priya Singh et al.   CELL

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

(2019) Di Hu et al.   ACTA NEUROPATHOLOGICA

ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response

(2019) Corey J. Anderson et al.   ACTA NEUROPATHOLOGICA

Mitochondrial ClpP-Mediated Proteolysis Induces Selective Cancer Cell Lethality

(2019) Jo Ishizawa et al.   CANCER CELL

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

(2019) Peng Wang et al.   PLoS Genetics

Neuronal XBP-1 Activates Intestinal Lysosomes to Improve Proteostasis in C. elegans

(2019) Soudabeh Imanikia et al.   CURRENT BIOLOGY

Translational Regulation of Non-autonomous Mitochondrial Stress Response Promotes Longevity

(2019) Jianfeng Lan et al.   Cell Reports

XBP-1 Remodels Lipid Metabolism to Extend Longevity

(2019) Soudabeh Imanikia et al.   Cell Reports

Improved mitochondrial stress response in long‐lived Snell dwarf mice

(2019) Ulas Ozkurede et al.   AGING CELL

Fibroblast Growth Factor 21 Drives Dynamics of Local and Systemic Stress Responses in Mitochondrial Myopathy with mtDNA Deletions

(2019) Saara Forsström et al.   Cell Metabolism

Ageing as a risk factor for neurodegenerative disease

(2019) Yujun Hou et al.   Nature Reviews Neurology

Dietary restriction improves proteostasis and increases life span through endoplasmic reticulum hormesis

(2019) Latika Matai et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An Early mtUPR: Redistribution of the Nuclear Transcription Factor Rox1 to Mitochondria Protects against Intramitochondrial Proteotoxic Aggregates

(2019) Daniel Poveda-Huertes et al.   MOLECULAR CELL

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

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

FSHR-1/GPCR Regulates the Mitochondrial Unfolded Protein Response in Caenorhabditis elegans

(2019) Sungjin Kim et al.   GENETICS

Developmental ROS individualizes organismal stress resistance and lifespan

(2019) Daphne Bazopoulou et al.   NATURE

Male lifespan extension with 17-α estradiol is linked to a sex-specific metabolomic response modulated by gonadal hormones in mice

(2018) Michael Garratt et al.   AGING CELL

Human Aging and Longevity Are Characterized by High Levels of Mitokines

(2018) Maria Conte et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

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

Quality Control in the Endoplasmic Reticulum: Crosstalk between ERAD and UPR pathways

(2018) Jiwon Hwang et al.   TRENDS IN BIOCHEMICAL SCIENCES

Correction: PGAM5 promotes lasting FoxO activation after developmental mitochondrial stress and extends lifespan in Drosophila

(2018) Martin Borch Jensen et al.   eLife

The different axes of the mammalian mitochondrial unfolded protein response

(2018) Christian Münch   BMC BIOLOGY

The Mitochondrial Unfolded Protein Response Is Mediated Cell-Non-autonomously by Retromer-Dependent Wnt Signaling

(2018) Qian Zhang et al.   CELL

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

Mitochondrial unfolded protein response transcription factor ATFS-1 promotes longevity in a long-lived mitochondrial mutant through activation of stress response pathways

(2018) Ziyun Wu et al.   BMC BIOLOGY

Food Perception Primes Hepatic ER Homeostasis via Melanocortin-Dependent Control of mTOR Activation

(2018) Claus Brandt et al.   CELL

SIRT3 Regulation Under Cellular Stress: Making Sense of the Ups and Downs

(2018) Joshua M. Marcus et al.   Frontiers in Neuroscience

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

From discovery of the CHOP axis and targeting ClpP to the identification of additional axes of the UPRmt driven by the estrogen receptor and SIRT3

(2017) Timothy C. Kenny et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Multi-omics analysis identifies ATF4 as a key regulator of the mitochondrial stress response in mammals

(2017) Pedro M. Quirós et al.   JOURNAL OF CELL BIOLOGY

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

(2017) Vijendra Sharma et al.   JOURNAL OF NEUROSCIENCE

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

Enhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity

(2017) Vincenzo Sorrentino et al.   NATURE

The mitochondrial UPR: mechanisms, physiological functions and implications in ageing

(2017) Tomer Shpilka et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The chromatin remodeling factor ISW-1 integrates organismal responses against nuclear and mitochondrial stress

(2017) Olli Matilainen et al.   Nature Communications

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

Mitochondrial Stress Restores the Heat Shock Response and Prevents Proteostasis Collapse during Aging

(2017) Johnathan Labbadia et al.   Cell Reports

Regulation of life span by the gut microbiota in the short-lived African turquoise killifish

(2017) Patrick Smith et al.   eLife

Amyotrophic lateral sclerosis

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

Parkinson disease

(2017) Werner Poewe et al.   Nature Reviews Disease Primers

Drosophila as a model for ageing

(2017) Matthew D.W. Piper et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Longer lifespan in male mice treated with a weakly estrogenic agonist, an antioxidant, an α-glucosidase inhibitor or a Nrf2-inducer

(2016) Randy Strong et al.   AGING CELL

Two Conserved Histone Demethylases Regulate Mitochondrial Stress-Induced Longevity

(2016) Carsten Merkwirth et al.   CELL

Mitochondrial Stress Induces Chromatin Reorganization to Promote Longevity and UPR mt

(2016) Ye Tian et al.   CELL

Neuroendocrine Coordination of Mitochondrial Stress Signaling and Proteostasis

(2016) Kristen M. Berendzen et al.   CELL

Homeostatic Responses Regulate Selfish Mitochondrial Genome Dynamics in C. elegans

(2016) Bryan L. Gitschlag et al.   Cell Metabolism

Neuropeptide signals cell non-autonomous mitochondrial unfolded protein response

(2016) Li-Wa Shao et al.   CELL RESEARCH

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

Growth differentiation factor 15 is a myomitokine governing systemic energy homeostasis

(2016) Hyo Kyun Chung et al.   JOURNAL OF CELL BIOLOGY

17α-Estradiol Alleviates Age-related Metabolic and Inflammatory Dysfunction in Male Mice Without Inducing Feminization

(2016) Michael B. Stout et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

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

Mitochondrial unfolded protein response controls matrix pre-RNA processing and translation

(2016) Christian Münch et al.   NATURE

Mitochondrial and nuclear DNA matching shapes metabolism and healthy ageing

(2016) Ana Latorre-Pellicer et al.   NATURE

Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response

(2016) Yi-Fan Lin et al.   NATURE

Protein misfolding in the endoplasmic reticulum as a conduit to human disease

(2016) Miao Wang et al.   NATURE

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

Intestinal IRE1 Is Required for Increased Triglyceride Metabolism and Longer Lifespan under Dietary Restriction

(2016) Nuno Miguel Luis et al.   Cell Reports

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

(2016) Lars Plate et al.   eLife

Enhancing NAD+salvage metabolism is neuroprotective in a PINK1 model of Parkinson's disease

(2016) Susann Lehmann et al.   Biology Open

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

(2015) Helois Radford et al.   ACTA NEUROPATHOLOGICA

PMT1 deficiency enhances basal UPR activity and extends replicative lifespan of Saccharomyces cerevisiae

(2015) Hong-Jing Cui et al.   AGE

Mitophagy and the mitochondrial unfolded protein response in neurodegeneration and bacterial infection

(2015) Mark W Pellegrino et al.   BMC BIOLOGY

Promoting Health and Longevity through Diet: From Model Organisms to Humans

(2015) Luigi Fontana et al.   CELL

Resveratrol reduces amyloid-beta (Aβ1–42)-induced paralysis through targeting proteostasis in an Alzheimer model of Caenorhabditis elegans

(2015) Charlotte Regitz et al.   EUROPEAN JOURNAL OF NUTRITION

The unpredictability of prolonged activation of stress response pathways

(2015) Lilian T. Lamech et al.   JOURNAL OF CELL BIOLOGY

Mitochondrial and Nuclear Accumulation of the Transcription Factor ATFS-1 Promotes OXPHOS Recovery during the UPRmt

(2015) Amrita M. Nargund et al.   MOLECULAR CELL

Repression of the Heat Shock Response Is a Programmed Event at the Onset of Reproduction

(2015) Johnathan Labbadia et al.   MOLECULAR CELL

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

(2015) Mercedes Prudencio et al.   NATURE NEUROSCIENCE

Increased GADD34 in oligodendrocytes in Alzheimer’s disease

(2015) Yasuyuki Honjo et al.   NEUROSCIENCE LETTERS

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

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

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

A Mutation in Caenorhabditis elegans NDUF-7 Activates the Mitochondrial Stress Response and Prolongs Lifespan via ROS and CED-4

(2015) Manish Rauthan et al.   G3-Genes Genomes Genetics

Mitochondrial and Cytoplasmic ROS Have Opposing Effects on Lifespan

(2015) Claire E. Schaar et al.   PLoS Genetics

PERK Limits Drosophila Lifespan by Promoting Intestinal Stem Cell Proliferation in Response to ER Stress

(2015) Lifen Wang et al.   PLoS Genetics

Complex IV-deficientSurf1−/−mice initiate mitochondrial stress responses

(2014) Daniel A. Pulliam et al.   BIOCHEMICAL JOURNAL

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

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

(2014) Liang Wu et al.   BRAIN RESEARCH

Axonally Synthesized ATF4 Transmits a Neurodegenerative Signal across Brain Regions

(2014) Jimena Baleriola et al.   CELL

The Intrinsic Apoptosis Pathway Mediates the Pro-Longevity Response to Mitochondrial ROS in C. elegans

(2014) Callista Yee et al.   CELL

Xbp1s in Pomc Neurons Connects ER Stress with Energy Balance and Glucose Homeostasis

(2014) Kevin W. Williams et al.   Cell Metabolism

Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

(2014) Nahid A Khan et al.   EMBO Molecular Medicine

The paradox of mitochondrial dysfunction and extended longevity

(2014) Erin Munkácsy et al.   EXPERIMENTAL GERONTOLOGY

Prolonged Fasting Identifies Heat Shock Protein 10 as a Sirtuin 3 Substrate

(2014) Zhongping Lu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial chaperone TRAP1 activates the mitochondrial UPR and extends healthspan in Drosophila

(2014) Rehan M. Baqri et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Sirtuin 3 interacts with Lon protease and regulates its acetylation status

(2014) Lara Gibellini et al.   MITOCHONDRION

Caenorhabditis elegans pathways that surveil and defend mitochondria

(2014) Ying Liu et al.   NATURE

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

Mitochondrial serine protease HTRA2 p.G399S in a kindred with essential tremor and Parkinson disease

(2014) Hilal Unal Gulsuner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Activation of the endoplasmic reticulum unfolded protein response by lipid disequilibrium without disturbed proteostasis in vivo

(2014) N. S. Hou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Getting RIDD of RNA: IRE1 in cell fate regulation

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

Proteostasis impairment in protein-misfolding and -aggregation diseases

(2014) Mark S. Hipp et al.   TRENDS IN CELL BIOLOGY

Activation of the mitochondrial unfolded protein response does not predict longevity in Caenorhabditis elegans

(2014) Christopher F. Bennett et al.   Nature Communications

Hypothalamic PGC-1α Protects Against High-Fat Diet Exposure by Regulating ERα

(2014) Eugenia Morselli et al.   Cell Reports

Lifespan Extension Conferred by Endoplasmic Reticulum Secretory Pathway Deficiency Requires Induction of the Unfolded Protein Response

(2014) Vyacheslav M. Labunskyy et al.   PLoS Genetics

XBP-1 Is a Cell-Nonautonomous Regulator of Stress Resistance and Longevity

(2013) Rebecca C. Taylor et al.   CELL

The Hallmarks of Aging

(2013) Carlos López-Otín et al.   CELL

The NAD+/Sirtuin Pathway Modulates Longevity through Activation of Mitochondrial UPR and FOXO Signaling

(2013) Laurent Mouchiroud et al.   CELL

Muscle Mitohormesis Promotes Longevity via Systemic Repression of Insulin Signaling

(2013) Edward Owusu-Ansah et al.   CELL

ER stress does not cause upregulation and activation of caspase-2 to initiate apoptosis

(2013) J J Sandow et al.   CELL DEATH AND DIFFERENTIATION

Stress-Regulated Translational Attenuation Adapts Mitochondrial Protein Import through Tim17A Degradation

(2013) T. Kelly Rainbolt et al.   Cell Metabolism

Characterization of global gene expression during assurance of lifespan extension by caloric restriction in budding yeast

(2013) Kyung-Mi Choi et al.   EXPERIMENTAL GERONTOLOGY

An enhanced integrated stress response ameliorates mutant SOD1-induced ALS

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

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

SirT3 Regulates the Mitochondrial Unfolded Protein Response

(2013) L. Papa et al.   MOLECULAR AND CELLULAR BIOLOGY

Mitonuclear protein imbalance as a conserved longevity mechanism

(2013) Riekelt H. Houtkooper et al.   NATURE

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

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

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

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

(2013) Soledad Matus et al.   PLoS One

The mitochondrial unfolded protein response activator ATFS-1 protects cells from inhibition of the mevalonate pathway

(2013) M. Rauthan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Stress-Independent Activation of XBP1s and/or ATF6 Reveals Three Functionally Diverse ER Proteostasis Environments

(2013) Matthew D. Shoulders et al.   Cell Reports

Neuronal ROS signaling rather than AMPK/sirtuin-mediated energy sensing links dietary restriction to lifespan extension

(2013) Sebastian Schmeisser et al.   Molecular Metabolism

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

Stress profiling of longevity mutants identifies Afg3 as a mitochondrial determinant of cytoplasmic mRNA translation and aging

(2012) Joe R. Delaney et al.   AGING CELL

ER stress inhibits neuronal death by promoting autophagy

(2012) Antoine Fouillet et al.   Autophagy

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

Impaired Insulin/IGF1 Signaling Extends Life Span by Promoting Mitochondrial L-Proline Catabolism to Induce a Transient ROS Signal

(2012) Kim Zarse et al.   Cell Metabolism

IRE1α Induces Thioredoxin-Interacting Protein to Activate the NLRP3 Inflammasome and Promote Programmed Cell Death under Irremediable ER Stress

(2012) Alana G. Lerner et al.   Cell Metabolism

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

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

Calorie Restriction and SIRT3 Trigger Global Reprogramming of the Mitochondrial Protein Acetylome

(2012) Alexander S. Hebert et al.   MOLECULAR CELL

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

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

IRE1  Cleaves Select microRNAs During ER Stress to Derepress Translation of Proapoptotic Caspase-2

(2012) J.-P. Upton et al.   SCIENCE

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

(2012) A. M. Nargund et al.   SCIENCE

Protective Coupling of Mitochondrial Function and Protein Synthesis via the eIF2α Kinase GCN-2

(2012) Brooke M. Baker et al.   PLoS Genetics

Endoplasmic reticulum stress in wake-active neurons progresses with aging

(2011) Nirinjini Naidoo et al.   AGING CELL

The Cell-Non-Autonomous Nature of Electron Transport Chain-Mediated Longevity

(2011) Jenni Durieux et al.   CELL

The ER stress factor XBP1s prevents amyloid-β neurotoxicity

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

Estrogen receptor mediates a distinct mitochondrial unfolded protein response

(2011) L. Papa et al.   JOURNAL OF CELL SCIENCE

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

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

FGF-21 as a biomarker for muscle-manifesting mitochondrial respiratory chain deficiencies: a diagnostic study

(2011) Anu Suomalainen et al.   LANCET NEUROLOGY

The failure to extend lifespan via disruption of complex II is linked to preservation of dynamic control of energy metabolism

(2011) Jujiao Kuang et al.   MITOCHONDRION

Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress

(2011) Ira Tabas et al.   NATURE CELL BIOLOGY

Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells

(2011) Giovanni Nardo et al.   PLoS One

Two modes of mitochondrial dysfunction lead independently to lifespan extension in Caenorhabditis elegans

(2010) Wen Yang et al.   AGING CELL

Inhibition of Respiration Extends C. elegans Life Span via Reactive Oxygen Species that Increase HIF-1 Activity

(2010) Seung-Jae Lee et al.   CURRENT BIOLOGY

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

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

(2010) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Mitochondrial electron transport chain dysfunction during development does not extend lifespan in Drosophila melanogaster

(2010) Michael Rera et al.   MECHANISMS OF AGEING AND DEVELOPMENT

The Matrix Peptide Exporter HAF-1 Signals a Mitochondrial UPR by Activating the Transcription Factor ZC376.7 in C. elegans

(2010) Cole M. Haynes et al.   MOLECULAR CELL

Activation of PERK Signaling Attenuates Aβ-Mediated ER Stress

(2010) Do Yeon Lee et al.   PLoS One

The Unfolded Protein Response Protects from Tau Neurotoxicity In Vivo

(2010) Carin A. Loewen et al.   PLoS One

Insulin/IGF-1 signaling mutants reprogram ER stress response regulators to promote longevity

(2010) S. Henis-Korenblit et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Mitochondrial Superoxide Signal Triggers Increased Longevity in Caenorhabditis elegans

(2010) Wen Yang et al.   PLOS BIOLOGY

Dysfunction of the unfolded protein response increases neurodegeneration in aged rat hippocampus following proteasome inhibition

(2009) María Paz Gavilán et al.   AGING CELL

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

The neuropathology of probable Alzheimer disease and mild cognitive impairment

(2009) Julie A. Schneider et al.   ANNALS OF NEUROLOGY

Is the oxidative stress theory of aging dead?

(2009) Viviana I. Pérez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Extension of Drosophila Life Span by RNAi of the Mitochondrial Respiratory Chain

(2009) Jeffrey M. Copeland et al.   CURRENT BIOLOGY

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

ATF6  induces XBP1-independent expansion of the endoplasmic reticulum

(2009) H. Bommiasamy et al.   JOURNAL OF CELL SCIENCE

Sirt3 blocks the cardiac hypertrophic response by augmenting Foxo3a-dependent antioxidant defense mechanisms in mice

(2009) Nagalingam R. Sundaresan et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Protein stability and resistance to oxidative stress are determinants of longevity in the longest-living rodent, the naked mole-rat

(2009) V. I. Perez et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging

(2009) A. Ben-Zvi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

HIF-1 Modulates Dietary Restriction-Mediated Lifespan Extension via IRE-1 in Caenorhabditis elegans

(2009) Di Chen et al.   PLoS Genetics

Protective effect against Parkinson's disease-related insults through the activation of XBP1

(2008) Megumi Sado et al.   BRAIN RESEARCH

Mitochondrial Protein Quality Control by the Proteasome Involves Ubiquitination and the Protease Omi

(2008) Susanne Radke et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aging Impairs the Unfolded Protein Response to Sleep Deprivation and Leads to Proapoptotic Signaling

(2008) N. Naidoo et al.   JOURNAL OF NEUROSCIENCE

Antihypertensive Drug Guanabenz Is Active In Vivo against both Yeast and Mammalian Prions

(2008) Déborah Tribouillard-Tanvier et al.   PLoS One

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Decreased enzyme activities of chaperones PDI and BiP in aged mouse livers

(2007) Jonathan E. Nuss et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS