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

The PERKs of mitochondria protection during stress: insights for PERK modulation in neurodegenerative and metabolic diseases

(2022) Liliana M. Almeida et al.   BIOLOGICAL REVIEWS

Prevalence and Incidence of Huntington's Disease: An Updated Systematic Review and Meta‐Analysis

(2022) Alex Medina et al.   MOVEMENT DISORDERS

Withaferin A Induces Heat Shock Response and Ameliorates Disease Progression in a Mouse Model of Huntington’s Disease

(2021) Tripti Joshi et al.   MOLECULAR NEUROBIOLOGY

The heat shock response, determined by QuantiGene multiplex, is impaired in HD mouse models and not caused by HSF1 reduction

(2021) Casandra Gomez-Paredes et al.   Scientific Reports

PC12 and THP-1 Cell Lines as Neuronal and Microglia Model in Neurobiological Research

(2021) Katarzyna Balon et al.   Applied Sciences-Basel

Synergistic effects of brain injury and aging: common mechanisms of proteostatic dysfunction

(2021) Janani Saikumar et al.   TRENDS IN NEUROSCIENCES

Cell Reprogramming to Model Huntington’s Disease: A Comprehensive Review

(2021) Ruth Monk et al.   Cells

Allosteric activation of Hsp70 reduces mutant huntingtin levels, the clustering of N-terminal fragments, and their nuclear accumulation

(2021) Brígida R. Pinho et al.   LIFE SCIENCES

Mitochondrial fission in Huntington's disease mouse striatum disrupts ER-mitochondria contacts leading to disturbances in Ca2+ efflux and Reactive Oxygen Species (ROS) homeostasis

(2020) Marta Cherubini et al.   NEUROBIOLOGY OF DISEASE

Mechanisms, regulation and functions of the unfolded protein response

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

A novel specific PERK activator reduces toxicity and extends survival in Huntington's disease models

(2020) Javier Ganz et al.   Scientific Reports

PC12 Cell Line: Cell Types, Coating of Culture Vessels, Differentiation and Other Culture Conditions

(2020) Benita Wiatrak et al.   Cells

Folding the Mitochondrial UPR into the Integrated Stress Response

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

Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

(2020) Sarah J. Tabrizi et al.   Nature Reviews Neurology

Classical swine fever virus employs the PERK- and IRE1-dependent autophagy for viral replication in cultured cells.

(2020) Erpeng Zhu et al.   Virulence

Opportunities and challenges for the use of induced pluripotent stem cells in modelling neurodegenerative disease

(2019) Yi-Ying Wu et al.   Open Biology

Huntingtin Lowering Strategies for Disease Modification in Huntington’s Disease

(2019) Sarah J. Tabrizi et al.   NEURON

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

PromISR-6, a Guanabenz Analogue, Improves Cellular Survival in an Experimental Model of Huntington’s Disease

(2019) Jeyapriya Rajameenakshi Sundaram et al.   ACS Chemical Neuroscience

The interplay between redox signalling and proteostasis in neurodegeneration: In vivo effects of a mitochondria-targeted antioxidant in Huntington's disease mice

(2019) Brígida R. Pinho et al.   FREE RADICAL BIOLOGY AND MEDICINE

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

(2018) Andrew Melber et al.   CELL RESEARCH

Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington’s disease

(2018) Xiao Zhou et al.   HUMAN MOLECULAR GENETICS

Striatal neurons directly converted from Huntington’s disease patient fibroblasts recapitulate age-associated disease phenotypes

(2018) Matheus B. Victor et al.   NATURE NEUROSCIENCE

Huntingtin Aggregation Impairs Autophagy, Leading to Argonaute-2 Accumulation and Global MicroRNA Dysregulation

(2018) Karolina Pircs et al.   Cell Reports

Targeting the proteostasis network in Huntington’s disease

(2018) Tânia R. Soares et al.   AGEING RESEARCH REVIEWS

Mitochondrial superoxide generation induces a parkinsonian phenotype in zebrafish and huntingtin aggregation in human cells

(2018) Brígida R. Pinho et al.   FREE RADICAL BIOLOGY AND MEDICINE

The HSP90 chaperone machinery

(2017) Florian H. Schopf et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

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

ER stress and the unfolded protein response in neurodegeneration

(2017) Claudio Hetz et al.   Nature Reviews Neurology

Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington’s disease

(2017) Rocio Gomez-Pastor et al.   Nature Communications

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

(2017) Johnathan Labbadia et al.   Cell Reports

Spatiotemporal Proteomic Profiling of Huntington’s Disease Inclusions Reveals Widespread Loss of Protein Function

(2017) Fabian Hosp et al.   Cell Reports

HSP90 recognizes the N-terminus of huntingtin involved in regulation of huntingtin aggregation by USP19

(2017) Wen-Tian He et al.   Scientific Reports

Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease

(2017) Elisabeth Singer et al.   Scientific Reports

HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models

(2017) Andreas Neueder et al.   Scientific Reports

Lipid Biosynthesis Coordinates a Mitochondrial-to-Cytosolic Stress Response

(2016) Hyun-Eui Kim et al.   CELL

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

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

The integrated stress response

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

Protopanaxtriol protects against 3-nitropropionic acid-induced oxidative stress in a rat model of Huntington's disease

(2015) Yan Gao et al.   ACTA PHARMACOLOGICA SINICA

Targeting Hsp90/Hsp70-Based Protein Quality Control for Treatment of Adult Onset Neurodegenerative Diseases

(2015) William B. Pratt et al.   Annual Review of Pharmacology and Toxicology

Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation

(2015) M.-T. El-Daher et al.   EMBO JOURNAL

Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease

(2015) S. Hwang et al.   EMBO Molecular Medicine

Rosiglitazone activation of PPARγ-dependent signaling is neuroprotective in mutant huntingtin expressing cells

(2015) Ming-Chang Chiang et al.   EXPERIMENTAL CELL RESEARCH

A common gene expression signature in Huntington’s disease patient brain regions

(2014) Andreas Neueder et al.   BMC Medical Genomics

Dexamethasone induces heat shock response and slows down disease progression in mouse and fly models of Huntington's disease

(2014) M. Maheshwari et al.   HUMAN MOLECULAR GENETICS

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1 

(2014) A. Hyrskyluoto et al.   HUMAN MOLECULAR GENETICS

Geldanamycin attenuates 3-nitropropionic acid-induced apoptosis and JNK activation through the expression of HSP 70 in striatal cells

(2014) YONG-JOON CHOI et al.   INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

Trehalose Reverses Cell Malfunction in Fibroblasts from Normal and Huntington's Disease Patients Caused by Proteosome Inhibition

(2014) Maria Angeles Fernandez-Estevez et al.   PLoS One

ER Stress-Induced eIF2-alpha Phosphorylation Underlies Sensitivity of Striatal Neurons to Pathogenic Huntingtin

(2014) Julia Leitman et al.   PLoS One

A Chaperome Subnetwork Safeguards Proteostasis in Aging and Neurodegenerative Disease

(2014) Marc Brehme et al.   Cell Reports

Modeling Huntington's disease with induced pluripotent stem cells

(2013) Julia A. Kaye et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

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

(2013) Julia Leitman et al.   Nature 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

Mechanism-Based Screen for G1/S Checkpoint Activators Identifies a Selective Activator of EIF2AK3/PERK Signalling

(2012) Simon R. Stockwell et al.   PLoS One

Impaired Heat Shock Response in Cells Expressing Full-Length Polyglutamine-Expanded Huntingtin

(2012) Sidhartha M. Chafekar et al.   PLoS One

An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity

(2012) G Cisbani et al.   Cell Death & Disease

Cell Fate Plug and Play: Direct Reprogramming and Induced Pluripotency

(2011) Stuart M. Chambers et al.   CELL

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

Novel mechanism of Hsp70 chaperone-mediated prevention of polyglutamine aggregates in a cellular model of huntington disease

(2011) I. V. Guzhova et al.   HUMAN MOLECULAR GENETICS

Changes in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin

(2011) P. Lajoie et al.   JOURNAL OF CELL SCIENCE

Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease

(2011) John Labbadia et al.   JOURNAL OF CLINICAL INVESTIGATION

BimEL as a possible molecular link between proteasome dysfunction and cell death induced by mutant huntingtin

(2010) Rebecca Leon et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

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

Modulation of Mutant Huntingtin N-Terminal Cleavage and Its Effect on Aggregation and Cell Death

(2010) Katrin Juenemann et al.   NEUROTOXICITY RESEARCH

Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

(2010) Hui Yang et al.   PLoS One

Modulation of Heat Shock Transcription Factor 1 as a Therapeutic Target for Small Molecule Intervention in Neurodegenerative Disease

(2010) Daniel W. Neef et al.   PLOS BIOLOGY

SCAMP5 Links Endoplasmic Reticulum Stress to the Accumulation of Expanded Polyglutamine Protein Aggregates via Endocytosis Inhibition

(2009) Jee-Yeon Noh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rrs1 Is Involved in Endoplasmic Reticulum Stress Response in Huntington Disease

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

Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease

(2009) K.J. Cho et al.   NEUROSCIENCE

Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor

(2008) Tomoyuki Yamanaka et al.   EMBO JOURNAL

Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins

(2008) Sami Reijonen et al.   EXPERIMENTAL CELL RESEARCH

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

(2008) M. L. Duennwald et al.   GENES & DEVELOPMENT

Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease

(2008) Wenzhen Duan et al.   NEUROBIOLOGY OF DISEASE