Targeting Hsp70 facilitated protein quality control for treatment of polyglutamine diseases

Genetics, Mechanisms, and Therapeutic Progress in Polyglutamine Spinocerebellar Ataxias

(2019) Ronald A.M. Buijsen et al.   Neurotherapeutics

Hsp70 and Hsp40 inhibit an inter-domain interaction necessary for transcriptional activity in the androgen receptor

(2019) Bahareh Eftekharzadeh et al.   Nature Communications

The coming of age of chaperone-mediated autophagy

(2018) Susmita Kaushik et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Protein Disaggregation in Multicellular Organisms

(2018) Nadinath B. Nillegoda et al.   TRENDS IN BIOCHEMICAL SCIENCES

Inhibitors and chemical probes for molecular chaperone networks

(2018) Jason E. Gestwicki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Androgen receptor polyglutamine expansion drives age-dependent quality control defects and muscle dysfunction

(2018) Samir R. Nath et al.   JOURNAL OF CLINICAL INVESTIGATION

A role for autophagy in Huntington's disease

(2018) Katherine R. Croce et al.   NEUROBIOLOGY OF DISEASE

Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks

(2018) Melanie Meister-Broekema et al.   Nature Communications

Hsp70 - a master regulator in protein degradation

(2017) María Rosario Fernández-Fernández et al.   FEBS LETTERS

Chaperone-mediated autophagy and endosomal microautophagy: Jointed by a chaperone

(2017) Kumsal Tekirdag et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

BAG3 Is a Modular, Scaffolding Protein that physically Links Heat Shock Protein 70 (Hsp70) to the Small Heat Shock Proteins

(2017) Jennifer N. Rauch et al.   JOURNAL OF MOLECULAR BIOLOGY

Regulation of heat shock transcription factors and their roles in physiology and disease

(2017) Rocio Gomez-Pastor et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Polyglutamine spinocerebellar ataxias — from genes to potential treatments

(2017) Henry L. Paulson et al.   NATURE REVIEWS NEUROSCIENCE

DNAJ Proteins in neurodegeneration: essential and protective factors

(2017) Christina Zarouchlioti et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Differential HspBP1 expression accounts for the greater vulnerability of neurons than astrocytes to misfolded proteins

(2017) Ting Zhao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Evolution of an intricate J-protein network driving protein disaggregation in eukaryotes

(2017) Nadinath B Nillegoda et al.   eLife

The Ubiquitination, Disaggregation and Proteasomal Degradation Machineries in Polyglutamine Disease

(2017) Samir R. Nath 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

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

Genetic interaction of hnRNPA2B1 and DNAJB6 in aDrosophilamodel of multisystem proteinopathy

(2016) Songqing Li et al.   HUMAN MOLECULAR GENETICS

Identification of a LargeDNAJB2Deletion in a Family with Spinal Muscular Atrophy and Parkinsonism

(2016) Elena Sanchez et al.   HUMAN MUTATION

Structural and Biological Interaction of hsc-70 Protein with Phosphatidylserine in Endosomal Microautophagy

(2016) Kateryna Morozova et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Compartment-Dependent Degradation of Mutant Huntingtin Accounts for Its Preferential Accumulation in Neuronal Processes

(2016) T. Zhao et al.   JOURNAL OF NEUROSCIENCE

Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts

(2016) Sofia Esteves et al.   MEDICINAL RESEARCH REVIEWS

The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model

(2016) Vaishali Kakkar et al.   MOLECULAR CELL

Modulation of Molecular Chaperones in Huntington’s Disease and Other Polyglutamine Disorders

(2016) Sara D. Reis et al.   MOLECULAR NEUROBIOLOGY

Hsp70 biases the folding pathways of client proteins

(2016) Ashok Sekhar et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autophagy wins the 2016 Nobel Prize in Physiology or Medicine: Breakthroughs in baker's yeast fuel advances in biomedical research

(2016) Beth Levine et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

In vivo aspects of protein folding and quality control

(2016) D. Balchin et al.   SCIENCE

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

NEDD4-mediated HSF1 degradation underlies α-synucleinopathy

(2015) Eunhee Kim et al.   HUMAN MOLECULAR GENETICS

Validation of the Hsp70-Bag3 Protein-Protein Interaction as a Potential Therapeutic Target in Cancer

(2015) X. Li et al.   MOLECULAR CANCER THERAPEUTICS

Human Hsp70 Disaggregase Reverses Parkinson’s-Linked α-Synuclein Amyloid Fibrils

(2015) Xuechao Gao et al.   MOLECULAR CELL

Action of the Hsp70 chaperone system observed with single proteins

(2015) João M. Nunes et al.   Nature Communications

Screening strategies to identify HSP70 modulators to treat Alzheimer’s disease

(2015) Jayanthi Repalli et al.   Drug Design Development and Therapy

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy

(2015) Paola Rusmini et al.   Scientific Reports

Huntington disease

(2015) Gillian P. Bates et al.   Nature Reviews Disease Primers

Hsp70–Bag3 Interactions Regulate Cancer-Related Signaling Networks

(2014) Teresa A. Colvin et al.   CANCER RESEARCH

Barcoding heat shock proteins to human diseases: looking beyond the heat shock response

(2014) V. Kakkar et al.   Disease Models & Mechanisms

A model in which heat shock protein 90 targets protein-folding clefts: Rationale for a new approach to neuroprotective treatment of protein folding diseases

(2014) William B Pratt et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

The Regulation of Autophagosome Dynamics by Huntingtin and HAP1 Is Disrupted by Expression of Mutant Huntingtin, Leading to Defective Cargo Degradation

(2014) Y. C. Wong et al.   JOURNAL OF NEUROSCIENCE

HSJ1-related hereditary neuropathies: Novel mutations and extended clinical spectrum

(2014) B. Gess et al.   NEUROLOGY

Age-Dependent Decrease in Chaperone Activity Impairs MANF Expression, Leading to Purkinje Cell Degeneration in Inducible SCA17 Mice

(2014) Su Yang et al.   NEURON

14-3-3 and aggresome formation: Implications in neurodegenerative diseases

(2014) Baohui Jia et al.   Prion

Potential function for the Huntingtin protein as a scaffold for selective autophagy

(2014) Joseph Ochaba et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Crystal structure of the nucleotide-binding domain of mortalin, the mitochondrial Hsp70 chaperone

(2014) Joseph Amick et al.   PROTEIN SCIENCE

Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding

(2014) Daniela Martino Roth et al.   PLOS BIOLOGY

Synthesis and Initial Evaluation of YM-08, a Blood-Brain Barrier Permeable Derivative of the Heat Shock Protein 70 (Hsp70) Inhibitor MKT-077, Which Reduces Tau Levels

(2013) Yoshinari Miyata et al.   ACS Chemical Neuroscience

Allosteric Heat Shock Protein 70 Inhibitors Rapidly Rescue Synaptic Plasticity Deficits by Reducing Aberrant Tau

(2013) Jose Abisambra et al.   BIOLOGICAL PSYCHIATRY

The Cotranslational Function of Ribosome-Associated Hsp70 in Eukaryotic Protein Homeostasis

(2013) Felix Willmund et al.   CELL

PolyQ Proteins Interfere with Nuclear Degradation of Cytosolic Proteins by Sequestering the Sis1p Chaperone

(2013) Sae-Hun Park et al.   CELL

DNAJB6 is a peptide-binding chaperone which can suppress amyloid fibrillation of polyglutamine peptides at substoichiometric molar ratios

(2013) Cecilia Månsson et al.   CELL STRESS & CHAPERONES

Transcriptional activation of TFEB/ZKSCAN3 target genes underlies enhanced autophagy in spinobulbar muscular atrophy

(2013) J. P. Chua et al.   HUMAN MOLECULAR GENETICS

A Platform to View Huntingtin Exon 1 Aggregation Flux in the Cell Reveals Divergent Influences from Chaperones hsp40 and hsp70

(2013) Angelique R. Ormsby et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Heat Shock Response Activation Exacerbates Inclusion Body Formation in a Cellular Model of Huntington Disease

(2013) Kirill Bersuker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cotranslational Response to Proteotoxic Stress by Elongation Pausing of Ribosomes

(2013) Botao Liu et al.   MOLECULAR CELL

Structure and function of Hip, an attenuator of the Hsp70 chaperone cycle

(2013) Zhuo Li et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy

(2013) Paola Rusmini et al.   NEUROBIOLOGY OF AGING

Huntington's disease: underlying molecular mechanisms and emerging concepts

(2013) John Labbadia et al.   TRENDS IN BIOCHEMICAL SCIENCES

Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110

(2013) Y Kuo et al.   Cell Death & Disease

Analogues of the Allosteric Heat Shock Protein 70 (Hsp70) Inhibitor, MKT-077, As Anti-Cancer Agents

(2013) Xiaokai Li et al.   ACS Medicinal Chemistry Letters

Ataxin-3 and Its E3 Partners: Implications for Machado–Joseph Disease

(2013) Thomas M. Durcan et al.   Frontiers in Neurology

Pharmacological Tuning of Heat Shock Protein 70 Modulates Polyglutamine Toxicity and Aggregation

(2012) Sidhartha M. Chafekar et al.   ACS Chemical Biology

Methylthioninium chloride (methylene blue) induces autophagy and attenuates tauopathy in vitro and in vivo

(2012) Erin E. Congdon et al.   Autophagy

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

(2012) John Labbadia et al.   BRAIN

Cysteine Reactivity Distinguishes Redox Sensing by the Heat-Inducible and Constitutive Forms of Heat Shock Protein 70

(2012) Yoshinari Miyata et al.   CHEMISTRY & BIOLOGY

Metazoan Hsp70 machines use Hsp110 to power protein disaggregation

(2012) Heike Rampelt et al.   EMBO JOURNAL

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

Methylene Blue Modulates Huntingtin Aggregation Intermediates and Is Protective in Huntington's Disease Models

(2012) E. M. Sontag et al.   JOURNAL OF NEUROSCIENCE

BAG3 mutations: another cause of giant axonal neuropathy

(2012) Fatima Jaffer et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

(2012) Adrienne M Wang et al.   Nature Chemical Biology

GRP78 counteracts cell death and protein aggregation caused by mutant huntingtin proteins

(2012) Yufeng Jiang et al.   NEUROSCIENCE LETTERS

Rhodacyanine Derivative Selectively Targets Cancer Cells and Overcomes Tamoxifen Resistance

(2012) John Koren et al.   PLoS One

Role of the CCAAT-Binding Protein NFY in SCA17 Pathogenesis

(2012) Li-Ching Lee et al.   PLoS One

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

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

PGC-1  Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function

(2012) T. Tsunemi et al.   Science Translational Medicine

Heme-Dependent Activation of Neuronal Nitric Oxide Synthase by Cytosol Is Due to an Hsp70-Dependent, Thioredoxin-Mediated Thiol–Disulfide Interchange in the Heme/Substrate Binding Cleft

(2011) Yoshihiro Morishima et al.   BIOCHEMISTRY

Neuronal expression of TATA box-binding protein containing expanded polyglutamine in knock-in mice reduces chaperone protein response by impairing the function of nuclear factor-Y transcription factor

(2011) Shanshan Huang et al.   BRAIN

Microautophagy of Cytosolic Proteins by Late Endosomes

(2011) Ranjit Sahu et al.   DEVELOPMENTAL CELL

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

Allosteric Drugs: The Interaction of Antitumor Compound MKT-077 with Human Hsp70 Chaperones

(2011) Aikaterini Rousaki et al.   JOURNAL OF MOLECULAR BIOLOGY

HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes

(2011) Alice S. Kwok et al.   JOURNAL OF NEUROCHEMISTRY

Constitutive Upregulation of Chaperone-Mediated Autophagy in Huntington's Disease

(2011) H. Koga et al.   JOURNAL OF NEUROSCIENCE

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3)

(2011) K. Seidel et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Co-Chaperone HSJ1a Dually Regulates the Proteasomal Degradation of Ataxin-3

(2011) Xue-Chao Gao et al.   PLoS One

Macroautophagy Is Regulated by the UPR–Mediator CHOP and Accentuates the Phenotype of SBMA Mice

(2011) Zhigang Yu et al.   PLoS Genetics

Binding of a Small Molecule at a Protein–Protein Interface Regulates the Chaperone Activity of Hsp70–Hsp40

(2010) Susanne Wisén et al.   ACS Chemical Biology

Autophagy: PolyQ toxic fragment turnover

(2010) Carlotta Duncan et al.   Autophagy

A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases

(2010) Valeria Crippa et al.   Autophagy

Proposal for a role of the Hsp90/Hsp70-based chaperone machinery in making triage decisions when proteins undergo oxidative and toxic damage

(2010) William B Pratt et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Inhibiting the ubiquitin–proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments

(2010) Xiang Li et al.   HUMAN MOLECULAR GENETICS

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

Inhibition of Hsp70 by Methylene Blue Affects Signaling Protein Function and Ubiquitination and Modulates Polyglutamine Protein Degradation

(2010) Adrienne M. Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Heat Shock Protein 70 (Hsp70) as an Emerging Drug Target

(2010) Christopher G. Evans et al.   JOURNAL OF MEDICINAL CHEMISTRY

A DNAJB Chaperone Subfamily with HDAC-Dependent Activities Suppresses Toxic Protein Aggregation

(2010) Jurre Hageman et al.   MOLECULAR CELL

The Heat Shock Response: Life on the Verge of Death

(2010) Klaus Richter et al.   MOLECULAR CELL

The kinetic parameters and energy cost of the Hsp70 chaperone as a polypeptide unfoldase

(2010) Sandeep K Sharma et al.   Nature Chemical Biology

Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease

(2010) Marta Martinez-Vicente et al.   NATURE NEUROSCIENCE

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

(2010) Harm H. Kampinga et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Chaperone-mediated autophagy dysfunction in the pathogenesis of neurodegeneration

(2010) Hiroshi Koga et al.   NEUROBIOLOGY OF DISEASE

Loss of Hsp70 Exacerbates Pathogenesis But Not Levels of Fibrillar Aggregates in a Mouse Model of Huntington's Disease

(2009) J. L. Wacker et al.   JOURNAL OF NEUROSCIENCE

Chemical Manipulation of Hsp70 ATPase Activity Regulates Tau Stability

(2009) U. K. Jinwal et al.   JOURNAL OF NEUROSCIENCE

Converging concepts of protein folding in vitro and in vivo

(2009) F Ulrich Hartl et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Guidelines for the nomenclature of the human heat shock proteins

(2008) Harm H. Kampinga et al.   CELL STRESS & CHAPERONES

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

(2008) Tomoyuki Yamanaka et al.   EMBO JOURNAL

CHIP deletion reveals functional redundancy of E3 ligases in promoting degradation of both signaling proteins and expanded glutamine proteins

(2008) Y. Morishima et al.   HUMAN MOLECULAR GENETICS

The Chaperone-Mediated Autophagy Receptor Organizes in Dynamic Protein Complexes at the Lysosomal Membrane

(2008) U. Bandyopadhyay et al.   MOLECULAR AND CELLULAR BIOLOGY

High-throughput screen for small molecules that modulate the ATPase activity of the molecular chaperone DnaK

(2007) Lyra Chang et al.   ANALYTICAL BIOCHEMISTRY