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

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

(2016) Miao Wang et al.   NATURE

A critical appraisal of the pathogenic protein spread hypothesis of neurodegeneration

(2016) Dominic M. Walsh et al.   NATURE REVIEWS NEUROSCIENCE

Mitochondrial dynamics and quality control in Huntington's disease

(2016) Pedro Guedes-Dias et al.   NEUROBIOLOGY OF DISEASE

Atomic structure of Hsp90-Cdc37-Cdk4 reveals that Hsp90 traps and stabilizes an unfolded kinase

(2016) K. A. Verba et al.   SCIENCE

The Biology of Proteostasis in Aging and Disease

(2015) Johnathan Labbadia et al.   Annual Review of Biochemistry

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

HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons

(2015) Pedro Guedes-Dias et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Reprint of “The mitochondrial permeability transition pore and its adaptive responses in tumor cells”

(2015) Andrea Rasola et al.   CELL CALCIUM

A Small Molecule Inhibitor of ATPase Activity of HSP70 Induces Apoptosis and Has Antitumor Activities

(2015) Sung-Kyun Ko et al.   CHEMISTRY & BIOLOGY

Role of the unfolded protein response, GRP78 and GRP94 in organ homeostasis

(2015) Genyuan Zhu et al.   JOURNAL OF CELLULAR PHYSIOLOGY

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

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

Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study

(2015) Heike Jacobi et al.   LANCET NEUROLOGY

Hsp90: Breaking the Symmetry

(2015) Matthias P. Mayer et al.   MOLECULAR CELL

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

(2015) Xuechao Gao et al.   MOLECULAR CELL

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

(2015) Nadinath B. Nillegoda et al.   NATURE

Proteostasis and aging

(2015) Susmita Kaushik et al.   NATURE MEDICINE

Structural basis of transcription initiation by RNA polymerase II

(2015) Sarah Sainsbury et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Toward therapeutic targets for SCA3: Insight into the role of Machado–Joseph disease protein ataxin-3 in misfolded proteins clearance

(2015) Xiaoling Li et al.   PROGRESS IN NEUROBIOLOGY

Molecular chaperones and neuronal proteostasis

(2015) Heather L. Smith et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Hsp90 interaction with clients

(2015) G. Elif Karagöz et al.   TRENDS IN BIOCHEMICAL SCIENCES

Molecular mechanism of Spinocerebellar Ataxia type 6: glutamine repeat disorder, channelopathy and transcriptional dysregulation. The multifaceted aspects of a single mutation

(2015) Paola Giunti et al.   Frontiers in Cellular Neuroscience

Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model

(2015) Warunee Dansithong et al.   PLoS Genetics

The Potential of Indole and a Synthetic Derivative for PolyQ Aggregation Reduction by Enhancement of the Chaperone and Autophagy Systems

(2014) Chih-Hsin Lin et al.   ACS Chemical Neuroscience

BAG3 induces the sequestration of proteasomal clients into cytoplasmic puncta

(2014) Melania Minoia et al.   Autophagy

Glucocorticoid Receptor Function Regulated by Coordinated Action of the Hsp90 and Hsp70 Chaperone Cycles

(2014) Elaine Kirschke et al.   CELL

A Quantitative Chaperone Interaction Network Reveals the Architecture of Cellular Protein Homeostasis Pathways

(2014) Mikko Taipale et al.   CELL

Hsp90-Tau Complex Reveals Molecular Basis for Specificity in Chaperone Action

(2014) G. Elif Karagöz et al.   CELL

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

Progress in the Discovery and Development of Heat Shock Protein 90 (Hsp90) Inhibitors

(2014) Rohit Bhat et al.   JOURNAL OF MEDICINAL CHEMISTRY

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

Slowing of neurodegeneration in Parkinson's disease and Huntington's disease: future therapeutic perspectives

(2014) Anthony H V Schapira et al.   LANCET

Modulation of the Hsp90 Chaperone Cycle by a Stringent Client Protein

(2014) Oliver Robin Lorenz et al.   MOLECULAR CELL

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

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

(2014) Su Yang et al.   NEURON

Chronic Treatment with 17-DMAG Improves Balance and Coordination in A New Mouse Model of Machado-Joseph Disease

(2014) Anabela Silva-Fernandes et al.   Neurotherapeutics

Hsp70 in cancer: back to the future

(2014) M Y Sherman et al.   ONCOGENE

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

Mitochondrial oxidative phosphorylation TRAP(1)ped in tumor cells

(2014) Andrea Rasola et al.   TRENDS IN CELL BIOLOGY

Proteostasis impairment in protein-misfolding and -aggregation diseases

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

Indole and synthetic derivative activate chaperone expression to reduce polyQ aggregation in SCA17 neuronal cell and slice culture models

(2014) Guey-Jen Lee-Chen et al.   Drug Design Development and Therapy

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

(2014) Nico P. Dantuma et al.   Frontiers in Molecular Neuroscience

Chemical Induction of Hsp70 Reduces α-Synuclein Aggregation in Neuroglioma Cells

(2013) Kiri Kilpatrick et al.   ACS Chemical 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

Molecular Chaperone Functions in Protein Folding and Proteostasis

(2013) Yujin E. Kim et al.   Annual Review of Biochemistry

HSPA8/HSC70 chaperone protein

(2013) François Stricher et al.   Autophagy

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

(2013) Jose Abisambra et al.   BIOLOGICAL PSYCHIATRY

Correlation between chemotype-dependent binding conformations of HSP90α/β and isoform selectivity—Implications for the structure-based design of HSP90α/β selective inhibitors for treating neurodegenerative diseases

(2013) Justin T. Ernst et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Second Cistron in CACNA1A Gene Encodes a Transcription Factor Mediating Cerebellar Development and SCA6

(2013) Xiaofei Du et al.   CELL

TRAP1 rescues PINK1 loss-of-function phenotypes

(2013) Li Zhang et al.   HUMAN MOLECULAR GENETICS

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

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

Inhibition of HSP90 molecular chaperones: moving into the clinic

(2013) Rocio Garcia-Carbonero et al.   LANCET ONCOLOGY

Chaperone machines for protein folding, unfolding and disaggregation

(2013) Helen Saibil   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Protein rescue from aggregates by powerful molecular chaperone machines

(2013) Shannon M. Doyle et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

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

A global view of Hsp90 functions

(2013) Gabriela Chiosis et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7

(2013) Udo Rüb et al.   PROGRESS IN NEUROBIOLOGY

Hsp70 chaperone dynamics and molecular mechanism

(2013) Matthias P. Mayer   TRENDS IN BIOCHEMICAL SCIENCES

The chaperone Hsp90: changing partners for demanding clients

(2013) Alina Röhl et al.   TRENDS IN BIOCHEMICAL SCIENCES

Huntington's disease: underlying molecular mechanisms and emerging concepts

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

Drosophila Trap1 protects against mitochondrial dysfunction in a PINK1/parkin model of Parkinson’s disease

(2013) A C Costa et al.   Cell Death & Disease

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

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

Analysis of the Tau-Associated Proteome Reveals That Exchange of Hsp70 for Hsp90 Is Involved in Tau Degradation

(2012) Andrea D. Thompson et al.   ACS Chemical Biology

Brain pathology of spinocerebellar ataxias

(2012) Kay Seidel et al.   ACTA NEUROPATHOLOGICA

Mitochondrial protein import: Common principles and physiological networks

(2012) Jan Dudek et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

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

(2012) John Labbadia et al.   BRAIN

Targeting the molecular chaperone heat shock protein 90 (HSP90): Lessons learned and future directions

(2012) David S. Hong et al.   CANCER TREATMENT REVIEWS

Hsp90 regulation of mitochondrial protein folding: from organelle integrity to cellular homeostasis

(2012) Dario C. Altieri   CELLULAR AND MOLECULAR LIFE SCIENCES

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

(2012) Yoshinari Miyata et al.   CHEMISTRY & BIOLOGY

Polyglutamine neurodegeneration: expanded glutamines enhance native functions

(2012) Harry T Orr   CURRENT OPINION IN GENETICS & DEVELOPMENT

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

(2012) S. T. Hansen et al.   HUMAN MOLECULAR GENETICS

HSPBs: Small proteins with big implications in human disease

(2012) Harm H. Kampinga et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

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

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

Cell biology of spinocerebellar ataxia

(2012) Harry T. Orr   JOURNAL OF CELL BIOLOGY

Development of a Grp94 inhibitor

(2012) Adam S. Duerfeldt et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Conservation between the RNA Polymerase I, II, and III Transcription Initiation Machineries

(2012) Alessandro Vannini et al.   MOLECULAR CELL

Spinocerebellar Ataxia Type 2: Clinical Presentation, Molecular Mechanisms, and Therapeutic Perspectives

(2012) J. J. Magaña et al.   MOLECULAR NEUROBIOLOGY

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

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

DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles

(2012) Kazushi Suzuki et al.   NEUROBIOLOGY OF DISEASE

Network Organization of the Huntingtin Proteomic Interactome in Mammalian Brain

(2012) Dyna I. Shirasaki et al.   NEURON

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

(2012) Yufeng Jiang et al.   NEUROSCIENCE LETTERS

Comprehensive review on the HSC70 functions, interactions with related molecules and involvement in clinical diseases and therapeutic potential

(2012) Tuoen Liu et al.   PHARMACOLOGY & THERAPEUTICS

Rhodacyanine Derivative Selectively Targets Cancer Cells and Overcomes Tamoxifen Resistance

(2012) John Koren et al.   PLoS One

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

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

TRAP1 Controls Mitochondrial Fusion/Fission Balance through Drp1 and Mff Expression

(2012) Hironori Takamura et al.   PLoS One

Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)

(2012) Masahisa Katsuno et al.   PROGRESS IN NEUROBIOLOGY

Chaperone-mediated autophagy: a unique way to enter the lysosome world

(2012) Susmita Kaushik et al.   TRENDS IN CELL BIOLOGY

PolyQ disease: misfiring of a developmental cell death program?

(2012) Elyse S. Blum et al.   TRENDS IN CELL BIOLOGY

Regulation of HSF1 Function in the Heat Stress Response: Implications in Aging and Disease

(2011) Julius Anckar et al.   Annual Review of Biochemistry

GRP94: An HSP90-like protein specialized for protein folding and quality control in the endoplasmic reticulum

(2011) Michal Marzec et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The Hsp90 chaperone machinery: Conformational dynamics and regulation by co-chaperones

(2011) Jing Li et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Evolution and function of diverse Hsp90 homologs and cochaperone proteins

(2011) Jill L. Johnson   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

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

TRAP1 and the proteasome regulatory particle TBP7/Rpt3 interact in the endoplasmic reticulum and control cellular ubiquitination of specific mitochondrial proteins

(2011) M R Amoroso et al.   CELL DEATH AND DIFFERENTIATION

Chemical Screens against a Reconstituted Multiprotein Complex: Myricetin Blocks DnaJ Regulation of DnaK through an Allosteric Mechanism

(2011) Lyra Chang et al.   CHEMISTRY & BIOLOGY

Targeting Heat Shock Proteins 70/90 and Proteasome for Cancer Therapy

(2011) R. E. Wang   CURRENT MEDICINAL CHEMISTRY

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

A Screen for Enhancers of Clearance Identifies Huntingtin as a Heat Shock Protein 90 (Hsp90) Client Protein

(2011) Barbara Baldo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

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

Ube2w and Ataxin-3 Coordinately Regulate the Ubiquitin Ligase CHIP

(2011) K. Matthew Scaglione et al.   MOLECULAR CELL

Molecular chaperones in protein folding and proteostasis

(2011) F. Ulrich Hartl et al.   NATURE

Small-molecule proteostasis regulators for protein conformational diseases

(2011) Barbara Calamini et al.   Nature Chemical Biology

Heat shock transcription factor 1 as a therapeutic target in neurodegenerative diseases

(2011) Daniel W. Neef et al.   NATURE REVIEWS DRUG DISCOVERY

Mitochondrial TRAP1 regulates the unfolded protein response in the endoplasmic reticulum

(2011) Kana Takemoto et al.   NEUROCHEMISTRY INTERNATIONAL

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

(2011) Xue-Chao Gao et al.   PLoS One

Toward understanding Machado–Joseph disease

(2011) Maria do Carmo Costa et al.   PROGRESS IN NEUROBIOLOGY

Small heat shock proteins and α-crystallins: dynamic proteins with flexible functions

(2011) Eman Basha et al.   TRENDS IN BIOCHEMICAL SCIENCES

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

On the mechanism of preprotein import by the mitochondrial presequence translocase

(2010) Martin van der Laan et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Chaperone-assisted degradation: multiple paths to destruction

(2010) Nadja Kettern et al.   BIOLOGICAL CHEMISTRY

Could successful (mitochondrial) networking help prevent Huntington's disease?

(2010) Jorge M. A. Oliveira et al.   EMBO Molecular Medicine

Mitochondrial bioenergetics and dynamics in Huntington’s disease: tripartite synapses and selective striatal degeneration

(2010) Jorge M. A. Oliveira   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

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

Nature and cause of mitochondrial dysfunction in Huntington’s disease: focusing on huntingtin and the striatum

(2010) Jorge M. A. Oliveira   JOURNAL OF NEUROCHEMISTRY

Disrupted Transforming Growth Factor-  Signaling in Spinal and Bulbar Muscular Atrophy

(2010) M. Katsuno et al.   JOURNAL OF NEUROSCIENCE

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

(2010) Jurre Hageman et al.   MOLECULAR CELL

Multifaceted Role of Heat Shock Protein 70 in Neurons

(2010) Tom Z. Lu et al.   MOLECULAR NEUROBIOLOGY

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

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

Mitochondrial protein import: from proteomics to functional mechanisms

(2010) Oliver Schmidt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

HSP90 at the hub of protein homeostasis: emerging mechanistic insights

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

Heat shock factors: integrators of cell stress, development and lifespan

(2010) Malin Åkerfelt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

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

17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy

(2010) Natalia B. Nedelsky et al.   NEURON

Nature's molecular sponges: Small heat shock proteins grow into their chaperone roles

(2010) S. J. Eyles et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein quality control in the ER: The recognition of misfolded proteins

(2010) Pekka Määttänen et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

GRP94 in ER quality control and stress responses

(2010) Davide Eletto et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

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

Partial Loss of Ataxin-1 Function Contributes to Transcriptional Dysregulation in Spinocerebellar Ataxia Type 1 Pathogenesis

(2010) Juan Crespo-Barreto et al.   PLoS Genetics

The carboxy-terminal fragment of α1A calcium channel preferentially aggregates in the cytoplasm of human spinocerebellar ataxia type 6 Purkinje cells

(2009) Taro Ishiguro et al.   ACTA NEUROPATHOLOGICA

A novel, small molecule inhibitor of Hsc70/Hsp70 potentiates Hsp90 inhibitor induced apoptosis in HCT116 colon carcinoma cells

(2009) Andrew J. Massey et al.   CANCER CHEMOTHERAPY AND PHARMACOLOGY

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3

(2009) Martin Gamerdinger et al.   EMBO JOURNAL

Deficit of heat shock transcription factor 1-heat shock 70 kDa protein 1A axis determines the cell death vulnerability in a model of spinocerebellar ataxia type 6

(2009) Li Li et al.   GENES TO CELLS

Transcriptional dysregulation of TrkA associates with neurodegeneration in spinocerebellar ataxia type 17

(2009) A. G. Shah et al.   HUMAN MOLECULAR GENETICS

Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy

(2009) Heather L. Montie et al.   HUMAN MOLECULAR GENETICS

Combinatorial drug design targeting multiple cancer signaling networks controlled by mitochondrial Hsp90

(2009) Byoung Heon Kang et al.   JOURNAL OF CLINICAL INVESTIGATION

HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease1

(2009) Joanna L. Howarth et al.   JOURNAL OF NEUROCHEMISTRY

Polyglutamine-Expanded Androgen Receptor Truncation Fragments Activate a Bax-Dependent Apoptotic Cascade Mediated by DP5/Hrk

(2009) J. E. Young et al.   JOURNAL OF NEUROSCIENCE

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

A Small Molecule Inhibitor of Inducible Heat Shock Protein 70

(2009) J. I-Ju Leu et al.   MOLECULAR CELL

Adapting the machine: adaptor proteins for Hsp100/Clp and AAA+ proteases

(2009) Janine Kirstein et al.   NATURE REVIEWS MICROBIOLOGY

The Hsp90 mosaic: a picture emerges

(2009) Matthias P Mayer et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation

(2009) An-Hsun Chou et al.   NEUROCHEMISTRY INTERNATIONAL

Phosphorylation of S776 and 14-3-3 Binding Modulate Ataxin-1 Interaction with Splicing Factors

(2009) Cesira de Chiara et al.   PLoS One

Atrophins’ emerging roles in development and neurodegenerative disease

(2008) Y. Shen et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

(2008) Tomoyuki Yamanaka et al.   EMBO JOURNAL

17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse

(2008) Keisuke Tokui et al.   HUMAN MOLECULAR GENETICS

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

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

(2008) Srikanth Ranganathan et al.   HUMAN MOLECULAR GENETICS

Polyglutamine Expansion Reduces the Association of TATA-binding Protein with DNA and Induces DNA Binding-independent Neurotoxicity

(2008) Meyer J. Friedman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Heat Shock Transcription Factor 1-activating Compounds Suppress Polyglutamine-induced Neurodegeneration through Induction of Multiple Molecular Chaperones

(2008) Nobuhiro Fujikake et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

(2008) Janghoo Lim et al.   NATURE

Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation

(2008) An-Hsun Chou et al.   NEUROBIOLOGY OF DISEASE

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis

(2008) Aislinn J. Williams et al.   NEUROBIOLOGY OF DISEASE

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE