Molecular Chaperones’ Potential against Defective Proteostasis of Amyotrophic Lateral Sclerosis

Differential roles for DNAJ isoforms in HTT-polyQ and FUS aggregation modulation revealed by chaperone screens

(2022) Kinneret Rozales et al.   Nature Communications

Proximity proteomics of C9orf72 dipeptide repeat proteins identifies molecular chaperones as modifiers of poly-GA aggregation

(2022) Feilin Liu et al.   Acta Neuropathologica Communications

Elevated constitutive expression of Hsp40 chaperone Sis1 reduces TDP-43 aggregation-induced oxidative stress in Ire1 pathway dependent-manner in yeast TDP-43 proteinopathy model of amyotrophic lateral sclerosis

(2022) Vidhya Bharathi et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Gel‐like inclusions of C‐terminal fragments of TDP‐43 sequester stalled proteasomes in neurons

(2022) Henrick Riemenschneider et al.   EMBO REPORTS

Survival analyses from the CENTAUR trial in amyotrophic lateral sclerosis: Evaluating the impact of treatment crossover on outcomes

(2022) Sabrina Paganoni et al.   MUSCLE & NERVE

Revisiting the Old Data of Heat Shock Protein 27 Expression in Squamous Cell Carcinoma: Enigmatic HSP27, More Than Heat Shock

(2022) Shutao Zheng et al.   Cells

Cellular Stress Induces Nucleocytoplasmic Transport Deficits Independent of Stress Granules

(2022) Joni Vanneste et al.   Biomedicines

Hsp70 exhibits a liquid-liquid phase separation ability and chaperones condensed FUS against amyloid aggregation

(2022) Yichen Li et al.   iScience

Genetics of amyotrophic lateral sclerosis: seeking therapeutic targets in the era of gene therapy

(2022) Naoki Suzuki et al.   JOURNAL OF HUMAN GENETICS

Systematic evaluation of genetic mutations in ALS: a population-based study

(2022) Maurizio Grassano et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

DDX5 mRNA-targeting Antisense Oligonucleotide as a new promising therapeutic in combating Castration-Resistant Prostate Cancer

(2022) Thi Khanh Le et al.   MOLECULAR THERAPY

HSPB8 Overexpression Ameliorates Cognitive Impairment in Diabetic Mice via Inhibiting NLRP3 Inflammation Activation

(2022) Yanmin Chang et al.   Oxidative Medicine and Cellular Longevity

Enhanced motor cortex output and disinhibition in asymptomatic female mice with C9orf72 genetic expansion

(2022) Sona Amalyan et al.   Cell Reports

Stress granule assembly in vivo is deficient in the CNS of mutant TDP-43 ALS mice

(2022) Alicia Dubinski et al.   HUMAN MOLECULAR GENETICS

Heat-shock chaperone HSPB1 regulates cytoplasmic TDP-43 phase separation and liquid-to-gel transition

(2022) Shan Lu et al.   NATURE CELL BIOLOGY

Identification of HSPB8 modulators counteracting misfolded protein accumulation in neurodegenerative diseases

(2022) Marta Chierichetti et al.   LIFE SCIENCES

C9orf72 regulates energy homeostasis by stabilizing mitochondrial complex I assembly

(2021) Tao Wang et al.   Cell Metabolism

FUS-dependent liquid–liquid phase separation is important for DNA repair initiation

(2021) Brunno R. Levone et al.   JOURNAL OF CELL BIOLOGY

Targeting the Mitochondrial Permeability Transition Pore to Prevent Age-Associated Cell Damage and Neurodegeneration

(2021) Andrew C. Kent et al.   Oxidative Medicine and Cellular Longevity

MARK2 phosphorylates eIF2α in response to proteotoxic stress

(2021) Yu-Ning Lu et al.   PLOS BIOLOGY

The Links between ALS and NF-κB

(2021) Emma Källstig et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

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

UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model

(2021) Kejing Zhang et al.   NEURON

The Role of Mitochondrial Dysfunction and ER Stress in TDP-43 and C9ORF72 ALS

(2021) Ruxandra Dafinca et al.   Frontiers in Cellular Neuroscience

Mitochondrion-Dependent Cell Death in TDP-43 Proteinopathies

(2021) Chantal B. Lucini et al.   Biomedicines

Hsp70 chaperones TDP-43 in dynamic, liquid-like phase and prevents it from amyloid aggregation

(2021) Jinge Gu et al.   CELL RESEARCH

Role of genetics in amyotrophic lateral sclerosis: a large cohort study in Chinese mainland population

(2021) Yong-Ping Chen et al.   JOURNAL OF MEDICAL GENETICS

Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway

(2021) Yen-Chen Lin et al.   NATURE NEUROSCIENCE

Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects

(2021) Jelena Scekic-Zahirovic et al.   Nature Communications

TDP-43 regulates GAD1 mRNA splicing and GABA signaling in Drosophila CNS

(2021) Giulia Romano et al.   Scientific Reports

HspB8 prevents aberrant phase transitions of FUS by chaperoning its folded RNA binding domain

(2021) Edgar E Boczek et al.   eLife

C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation

(2021) Nausicaa V Licata et al.   EMBO JOURNAL

Cytoplasmic TDP-43 impairs the activity of the ubiquitin-proteasome system

(2021) Peng Yin et al.   EXPERIMENTAL NEUROLOGY

The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis

(2021) Joni Vanneste et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mutation screening of the DNAJC7 gene in Japanese patients with sporadic amyotrophic lateral sclerosis

(2021) Genki Tohnai et al.   NEUROBIOLOGY OF AGING

ALS-linked cytoplasmic FUS assemblies are compositionally different from physiological stress granules and sequester hnRNPA3, a novel modifier of FUS toxicity

(2021) Haiyan An et al.   NEUROBIOLOGY OF DISEASE

A C. elegans model of C9orf72-associated ALS/FTD uncovers a conserved role for eIF2D in RAN translation

(2021) Yoshifumi Sonobe et al.   Nature Communications

TDP-43 and ER Stress in Neurodegeneration: Friends or Foes?

(2021) Lorena de Mena et al.   Frontiers in Molecular Neuroscience

Depending on the stress, histone deacetylase inhibitors act as heat shock protein co-inducers in motor neurons and potentiate arimoclomol, exerting neuroprotection through multiple mechanisms in ALS models

(2020) Rachel Kuta et al.   CELL STRESS & CHAPERONES

Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD

(2020) Nadja S. Andrade et al.   Molecular Neurodegeneration

ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs

(2020) Yueh-Lin Tsai et al.   GENES & DEVELOPMENT

Functional diversity between HSP70 paralogs caused by variable interactions with specific co-chaperones

(2020) Despina Serlidaki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp27 chaperones FUS phase separation under the modulation of stress-induced phosphorylation

(2020) Zhenying Liu et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes

(2020) James N. Sleigh et al.   Cell Reports

Four novel Optineurin mutations in patients with sporadic amyotrophic lateral sclerosis in mainland China

(2020) Lu Yang et al.   NEUROBIOLOGY OF AGING

C9orf72-associated SMCR8 protein binds in the ubiquitin pathway and with proteins linked with neurological disease

(2020) John L. Goodier et al.   Acta Neuropathologica Communications

Rapid progressive ALS in a patient with a DNAJC7 loss-of-function mutation

(2020) Kang-Yang Jih et al.   Neurology-Genetics

Molecular and pharmacological chaperones for SOD1

(2020) Gareth S.A. Wright   BIOCHEMICAL SOCIETY TRANSACTIONS

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

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

(2020) Chien-Hsiung Yu et al.   CELL

Amyotrophic lateral sclerosis associated mislocalisation of TDP‐43 to the cytoplasm causes cortical hyperexcitability and reduced excitatory neurotransmission in the motor cortex

(2020) MS. Dyer et al.   JOURNAL OF NEUROCHEMISTRY

Withaferin-A Treatment Alleviates TAR DNA-Binding Protein-43 Pathology and Improves Cognitive Function in a Mouse Model of FTLD

(2020) Sunny Kumar et al.   Neurotherapeutics

Mutations of DNAJC7 are rare in Chinese amyotrophic lateral sclerosis patients

(2020) Xiaohan Sun et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Ubiquitin homeostasis is disrupted in TDP-43 and FUS cell models of ALS

(2020) Natalie E. Farrawell et al.   iScience

C-terminus of Hsp70 Interacting Protein (CHIP) and Neurodegeneration: Lessons from the Bench and Bedside

(2020) Sivakami Mylvaganam et al.   Current Neuropharmacology

The Regulation of Astrocytic Glutamate Transporters in Health and Neurodegenerative Diseases

(2020) Alison C. Todd et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Hsp40 proteins phase separate to chaperone the assembly and maintenance of membraneless organelles

(2020) Jinge Gu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Very Early Involvement of Innate Immunity in Peripheral Nerve Degeneration in SOD1-G93A Mice

(2020) Daniela Francesca Angelini et al.   Frontiers in Immunology

Caffeic acid phenethyl ester protects against oxidative stress and dampens inflammation via heme oxygenase 1

(2019) Alexandra Stähli et al.   International Journal of Oral Science

Functional Modules of the Proteostasis Network

(2019) Gopal G. Jayaraj et al.   Cold Spring Harbor Perspectives in Biology

Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects

(2019) Joy Mitra et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

(2019) Jeannie Chew et al.   Molecular Neurodegeneration

FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy

(2019) Lara Marrone et al.   ACTA NEUROPATHOLOGICA

Polyphenolic flavonoid (Myricetin) upregulated proteasomal degradation mechanisms: Eliminates neurodegenerative proteins aggregation

(2019) Vibhuti Joshi et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Nuclear Phospho-SOD1 Protects DNA from Oxidative Stress Damage in Amyotrophic Lateral Sclerosis

(2019) Matteo Bordoni et al.   Journal of Clinical Medicine

HSP70/DNAJA3 chaperone/cochaperone regulates NF-κB activity in immune responses

(2019) Kohei Kumada et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Regional differences in the inflammatory and heat shock response in glia: implications for ALS

(2019) Benjamin E. Clarke et al.   CELL STRESS & CHAPERONES

Pathophysiology and Diagnosis of ALS: Insights from Advances in Neurophysiological Techniques

(2019) Mehdi A. J. van den Bos et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Proteostasis and ALS: protocol for a phase II, randomised, double-blind, placebo-controlled, multicentre clinical trial for colchicine in ALS (Co-ALS)

(2019) Jessica Mandrioli et al.   BMJ Open

Aβ42 fibril formation from predominantly oligomeric samples suggests a link between oligomer heterogeneity and fibril polymorphism

(2019) Christine Xue et al.   Royal Society Open Science

Histamine Is an Inducer of the Heat Shock Response in SOD1-G93A Models of ALS

(2019) Savina Apolloni et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins

(2019) Vandana Deora et al.   GLIA

Neuroprotective effects of increasing levels of HSP70 against neuroinflammation in Parkinson's disease model by inhibition of NF-κB and STAT3

(2019) Haining Li et al.   LIFE SCIENCES

Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein

(2019) Sali M. K. Farhan et al.   NATURE NEUROSCIENCE

C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport

(2019) Joni Vanneste et al.   Scientific Reports

Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

(2019) Oliver H. Tam et al.   Cell Reports

Cytoplasmic Restriction of Mutated SOD1 Impairs the DNA Repair Process in Spinal Cord Neurons

(2019) Li et al.   Cells

Mutant C9orf72 human iPSC‐derived astrocytes cause non‐cell autonomous motor neuron pathophysiology

(2019) Chen Zhao et al.   GLIA

Stress granule mediated protein aggregation and underlying gene defects in the FTD-ALS spectrum

(2019) Yalda Baradaran-Heravi et al.   NEUROBIOLOGY OF DISEASE

TDP-43 knockdown causes innate immune activation via protein kinase R in astrocytes

(2019) Thomas J. LaRocca et al.   NEUROBIOLOGY OF DISEASE

Mechanisms of Immune Activation by c9orf72-Expansions in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

(2019) Kyle J. Trageser et al.   Frontiers in Neuroscience

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

(2018) Ke Zhang et al.   CELL

Amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease (AD) are characterised by differential activation of ER stress pathways: focus on UPR target genes

(2018) L. Montibeller et al.   CELL STRESS & CHAPERONES

HSPB8 and BAG3 cooperate to promote spatial sequestration of ubiquitinated proteins and coordinate the cellular adaptive response to proteasome insufficiency

(2018) Solenn M. Guilbert et al.   FASEB JOURNAL

Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha

(2018) Azadeh Kia et al.   GLIA

Proteasome-mediated proteostasis: Novel medicinal and pharmacological strategies for diseases

(2018) Ribhav Mishra et al.   MEDICINAL RESEARCH REVIEWS

Astrocyte elevated gene-1 is a novel regulator of astrogliosis and excitatory amino acid transporter-2 via interplaying with nuclear factor-κB signaling in astrocytes from amyotrophic lateral sclerosis mouse model with hSOD1 G93A mutation

(2018) Xiang Yin et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

Translation of dipeptide repeat proteins from the C9ORF72 expanded repeat is associated with cellular stress

(2018) Yoshifumi Sonobe et al.   NEUROBIOLOGY OF DISEASE

Randomized, double-blind, placebo-controlled trial of arimoclomol in rapidly progressiveSOD1ALS

(2018) Michael Benatar et al.   NEUROLOGY

Overexpression of a conserved HSP40 chaperone reduces toxicity of several neurodegenerative disease proteins

(2018) Sei-Kyoung Park et al.   Prion

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

(2018) Yousra Khalfallah et al.   Scientific Reports

Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity

(2018) Elke Bogaert et al.   Cell Reports

Hsp70 Suppresses Mitochondrial Reactive Oxygen Species and Preserves Pulmonary Microvascular Barrier Integrity Following Exposure to Bacterial Toxins

(2018) Xueyi Li et al.   Frontiers in Immunology

Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

(2018) Daniel A. Mordes et al.   Acta Neuropathologica Communications

DNAJC5 facilitates USP19-dependent unconventional secretion of misfolded cytosolic proteins

(2018) Yue Xu et al.   Cell Discovery

Molecular chaperone HSP70 prevents formation of inclusion bodies of the 25-kDa C-terminal fragment of TDP-43 by preventing aggregate accumulation

(2018) Akira Kitamura et al.   CELL STRESS & CHAPERONES

Heat Shock Protein B8 (HSPB8) Reduces Oxygen-Glucose Deprivation/Reperfusion Injury via the Induction of Mitophagy

(2018) Fazhao Li et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

Secretion of misfolded cytosolic proteins from mammalian cells is independent of chaperone-mediated autophagy

(2018) Juhyung Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

C9orf72 Dipeptide Repeats Cause Selective Neurodegeneration and Cell-Autonomous Excitotoxicity inDrosophilaGlutamatergic Neurons

(2018) Wangchao Xu et al.   JOURNAL OF NEUROSCIENCE

Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis

(2018) Haibo Wang et al.   Nature Communications

Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells

(2018) Maria Elena Cicardi et al.   Scientific Reports

Misfolded SOD1 pathology in sporadic Amyotrophic Lateral Sclerosis

(2018) Bastien Paré et al.   Scientific Reports

Microglial overexpression of fALS-linked mutant SOD1 induces SOD1 processing impairment, activation and neurotoxicity and is counteracted by the autophagy inducer trehalose

(2018) Francesca Massenzio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Synthesis and evaluation of esterified Hsp70 agonists in cellular models of protein aggregation and folding

(2018) Annette N. Chiang et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Rapidly progressive amyotrophic lateral sclerosis is associated with microglial reactivity and small heat shock protein expression in reactive astrocytes

(2018) R. P. Gorter et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Astrocyte-Mediated Neuromodulatory Regulation in Preclinical ALS: A Metadata Analysis

(2018) Kathleen Jordan et al.   Frontiers in Cellular Neuroscience

The Hsp70 co-chaperone Ydj1/HDJ2 regulates ribonucleotide reductase activity

(2018) Isaac T. Sluder et al.   PLoS Genetics

HSPB1 mutations causing hereditary neuropathy in humans disrupt non-cell autonomous protection of motor neurons

(2017) Patrick L. Heilman et al.   EXPERIMENTAL NEUROLOGY

Hsp70's RNA-binding and mRNA-stabilizing activities are independent of its protein chaperone functions

(2017) Aparna Kishor et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The small heat shock protein Hsp27: Present understanding and future prospects

(2017) Manish Kumar Singh et al.   JOURNAL OF THERMAL BIOLOGY

Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial

(2017) Koji Abe et al.   LANCET NEUROLOGY

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

The evolving genetic risk for sporadic ALS

(2017) Summer B. Gibson et al.   NEUROLOGY

Aberrant distributions of nuclear pore complex proteins in ALS mice and ALS patients

(2017) Jingwei Shang et al.   NEUROSCIENCE

The role of mitochondria in amyotrophic lateral sclerosis

(2017) Emma F. Smith et al.   NEUROSCIENCE LETTERS

Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrity

(2017) Jung-A. A. Woo et al.   Nature Communications

Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis

(2017) Sei-Kyoung Park et al.   PLoS Genetics

Nuclear export of misfolded SOD1 mediated by a normally buried NES-like sequence reduces proteotoxicity in the nucleus

(2017) Yongwang Zhong et al.   eLife

Decreased Levels of Foldase and Chaperone Proteins Are Associated with an Early-Onset Amyotrophic Lateral Sclerosis

(2017) Melania Filareti et al.   Frontiers in Molecular Neuroscience

The Role of the Multifunctional BAG3 Protein in Cellular Protein Quality Control and in Disease

(2017) Elisabeth Stürner et al.   Frontiers in Molecular Neuroscience

Protein Quality Control by Molecular Chaperones in Neurodegeneration

(2017) Aaron Ciechanover et al.   Frontiers in Neuroscience

Amyotrophic lateral sclerosis

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

Highly Selective Activation of Heat Shock Protein 70 by Allosteric Regulation Provides an Insight into Efficient Neuroinflammation Inhibition

(2017) Li-Chao Wang et al.   EBioMedicine

The molecular chaperones DNAJB6 and Hsp70 cooperate to suppress α-synuclein aggregation

(2017) Francesco A. Aprile et al.   Scientific Reports

The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis

(2016) Han-Jou Chen et al.   BRAIN

Heat Shock Protein (HSP) Drug Discovery and Development: Targeting Heat Shock Proteins in Disease

(2016) Liza Shrestha et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

DnaJ/Hsc70 chaperone complexes control the extracellular release of neurodegenerative‐associated proteins

(2016) Sarah N Fontaine et al.   EMBO JOURNAL

An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing

(2016) Hisashi Ito et al.   INTERNAL MEDICINE

A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism

(2016) Massimo Ganassi et al.   MOLECULAR CELL

Unconventional secretion of misfolded proteins promotes adaptation to proteasome dysfunction in mammalian cells

(2016) Jin-Gu Lee et al.   NATURE CELL BIOLOGY

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

HSP27 Alleviates Cardiac Aging in Mice via a Mechanism Involving Antioxidation and Mitophagy Activation

(2016) Shenglan Lin et al.   Oxidative Medicine and Cellular Longevity

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases

(2016) Valeria Crippa et al.   Scientific Reports

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion

(2015) Q. Xia et al.   EMBO JOURNAL

TDP-43 activates microglia through NF-κB and NLRP3 inflammasome

(2015) Weihua Zhao et al.   EXPERIMENTAL NEUROLOGY

BiP and Its Nucleotide Exchange Factors Grp170 and Sil1: Mechanisms of Action and Biological Functions

(2015) Julia Behnke et al.   JOURNAL OF MOLECULAR BIOLOGY

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

(2015) Nadinath B. Nillegoda et al.   NATURE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

(2015) Tetsuro Murakami et al.   NEURON

Coupling of Ribostasis and Proteostasis: Hsp70 Proteins in mRNA Metabolism

(2015) Robert W. Walters et al.   TRENDS IN BIOCHEMICAL SCIENCES

Mitochondrial Heat Shock Protein Machinery Hsp70/Hsp40 Is Indispensable for Proper Mitochondrial DNA Maintenance and Replication

(2015) Jiří Týč et al.   mBio

Autophagy dysregulation by mutant fused in sarcoma—implications for amyotrophic lateral sclerosis

(2015) K Y Soo et al.   Cell Death & Disease

Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain

(2015) Margaret M. P. Pearce et al.   Nature Communications

Optineurin mutations in patients with sporadic amyotrophic lateral sclerosis in China

(2015) Chengyu Li et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation

(2015) Jingyan Zhu et al.   PLoS One

Protective effect of geranylgeranylacetone via enhanced induction of HSPB1 and HSPB8 in mitochondria of the failing heart following myocardial infarction in rats

(2014) Tetsuro Marunouchi et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS

(2014) Mai Yamakawa et al.   HUMAN MOLECULAR GENETICS

The Functional Landscape of Hsp27 Reveals New Cellular Processes such as DNA Repair and Alternative Splicing and Proposes Novel Anticancer Targets

(2014) Maria Katsogiannou et al.   MOLECULAR & CELLULAR PROTEOMICS

Microglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis

(2014) Ashley E. Frakes et al.   NEURON

Mitochondrial Reactive Oxygen Species (ROS) and ROS-Induced ROS Release

(2014) Dmitry B. Zorov et al.   PHYSIOLOGICAL REVIEWS

A Splicing Mutation in the Novel Mitochondrial Protein DNAJC11 Causes Motor Neuron Pathology Associated with Cristae Disorganization, and Lymphoid Abnormalities in Mice

(2014) Fotis Ioakeimidis et al.   PLoS One

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

(2013) Jianbin Tong et al.   EMBO JOURNAL

Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones

(2013) Maria Udan-Johns et al.   HUMAN MOLECULAR GENETICS

The DNAJB6 and DNAJB8 Protein Chaperones Prevent Intracellular Aggregation of Polyglutamine Peptides

(2013) Judith Gillis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS

(2013) Peter E.A. Ash et al.   NEURON

Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2013) Sergey S. Novoselov et al.   PLoS One

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

(2013) David MA Mann et al.   Acta Neuropathologica Communications

Novel inhibitors of heat shock protein Hsp70-mediated luciferase refolding that bind to DnaJ

(2012) Joel A. Cassel et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72

(2012) Johnathan Cooper-Knock et al.   BRAIN

Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels

(2012) Masato Kato et al.   CELL

The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro

(2012) Justin J. Yerbury et al.   CELL STRESS & CHAPERONES

Mitochondrial chaperone DnaJA3 induces Drp1-dependent mitochondrial fragmentation

(2012) Adam N. Elwi et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Nuclear Localization of Human SOD1 and Mutant SOD1-Specific Disruption of Survival Motor Neuron Protein Complex in Transgenic Amyotrophic Lateral Sclerosis Mice

(2012) Barry Gertz et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase

(2012) Manal A. Farg et al.   NEUROBIOLOGY OF AGING

E6-AP association promotes SOD1 aggresomes degradation and suppresses toxicity

(2012) Amit Mishra et al.   NEUROBIOLOGY OF AGING

Protein Folding at the Exit Tunnel

(2011) Daria V. Fedyukina et al.   Annual Review of Biophysics

Transduced HSP27 protein protects neuronal cell death by enhancing FALS-associated SOD1 mutant activity

(2011) Jae-Jin An et al.   BMB Reports

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

(2011) Mariely DeJesus-Hernandez et al.   NEURON

Caffeic acid phenethyl ester extends survival of a mouse model of amyotrophic lateral sclerosis

(2011) C.V. Fontanilla et al.   NEUROSCIENCE

Misfolded protein aggregates: Mechanisms, structures and potential for disease transmission

(2011) Ines Moreno-Gonzalez et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Cellular Strategies of Protein Quality Control

(2011) B. Chen et al.   Cold Spring Harbor Perspectives in Biology

Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy

(2011) Kazuo Fushimi et al.   Protein & Cell

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Abnormal exocytotic release of glutamate in a mouse model of amyotrophic lateral sclerosis

(2010) Marco Milanese et al.   JOURNAL OF NEUROCHEMISTRY

HSP90 at the hub of protein homeostasis: emerging mechanistic insights

(2010) Mikko Taipale et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

(2010) Liqun Liu-Yesucevitz 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

Heat shock protein 27 in neuronal survival and neurite outgrowth

(2009) Danielle E. Read et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

(2009) Brian S. Johnson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activation of Gene Transcription by Heat Shock Protein 27 May Contribute to Its Neuronal Protection

(2009) Meyer J. Friedman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Structural and Functional Diversities between Members of the Human HSPB, HSPH, HSPA, and DNAJ Chaperone Families†

(2008) Michel J. Vos et al.   BIOCHEMISTRY

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

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

Hsp27 Regulates Pro-Inflammatory Mediator Release in Keratinocytes by Modulating NF-κB Signaling

(2007) Runa Sur et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression

(2007) Paul S. Sharp et al.   NEUROBIOLOGY OF DISEASE