Hsp104 Suppresses Polyglutamine-Induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model

A screen of apoptosis and senescence regulatory genes for life span effects when over-expressed in Drosophila

(2016) Jie Shen et al.   Aging-US

Correction: Progress in gene therapy for neurological disorders

(2013) Michele Simonato et al.   Nature Reviews Neurology

Gene Therapy for Misfolding Protein Diseases of the Central Nervous System

(2013) Waldy San Sebastian et al.   Neurotherapeutics

Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients

(2012) Morgan E. DeSantis et al.   CELL

Hsp104 Drives “Protein-Only” Positive Selection of Sup35 Prion Strains Encoding Strong [PSI+]

(2012) Morgan E. DeSantis et al.   CHEMISTRY & BIOLOGY

Metazoan Hsp70 machines use Hsp110 to power protein disaggregation

(2012) Heike Rampelt et al.   EMBO JOURNAL

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

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

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models

(2012) Maria Armakola et al.   NATURE GENETICS

Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans

(2012) Martin L. Duennwald et al.   PLOS BIOLOGY

Retargeted Clostridial Neurotoxins as Novel Agents for Treating Chronic Diseases

(2011) Felix L. Yeh et al.   BIOCHEMISTRY

The Josephin Domain Determines the Morphological and Mechanical Properties of Ataxin-3 Fibrils

(2011) Laura Masino et al.   BIOPHYSICAL JOURNAL

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

Aberrant protein interactions in amyloid disease

(2011) R. Martin Vabulas et al.   CELL CYCLE

The Machado-Joseph disease deubiquitylase ATX-3 couples longevity and proteostasis

(2011) Kirsten Kuhlbrodt et al.   NATURE CELL BIOLOGY

Dominant prion mutants induce curing through pathways that promote chaperone-mediated disaggregation

(2011) Susanne DiSalvo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Machado-Joseph Disease: from first descriptions to new perspectives

(2011) Conceição Bettencourt et al.   Orphanet Journal of Rare Diseases

The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System

(2011) James Shorter   PLoS One

Amyloid in neurodegenerative diseases: Friend or foe?

(2011) Katie J. Wolfe et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

A Yeast Model of FUS/TLS-Dependent Cytotoxicity

(2011) Shulin Ju et al.   PLOS BIOLOGY

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun et al.   PLOS BIOLOGY

Prion-like disorders: blurring the divide between transmissibility and infectivity

(2010) M. Cushman et al.   JOURNAL OF CELL SCIENCE

TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97

(2010) G. P. Ritson et al.   JOURNAL OF NEUROSCIENCE

Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

(2010) Ginny Marie Harris et al.   PLoS One

A Systematic Survey Identifies Prions and Illuminates Sequence Features of Prionogenic Proteins

(2009) Simon Alberti et al.   CELL

The role of molecular chaperones in human misfolding diseases

(2009) Sarah A. Broadley et al.   FEBS LETTERS

Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3

(2009) Joonil Jung et al.   HUMAN MOLECULAR GENETICS

Motor Mechanism for Protein Threading through Hsp104

(2009) Petra Wendler et al.   MOLECULAR CELL

Botulinum neurotoxins for post-stroke spasticity in adults: A systematic review

(2009) Antonio Emanuele Elia et al.   MOVEMENT DISORDERS

Increased transcript diversity: novel splicing variants of Machado–Joseph Disease gene (ATXN3)

(2009) Conceição Bettencourt et al.   NEUROGENETICS

Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity

(2009) Y. Nekooki-Machida et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Botulinum toxin for the management of adult patients with upper motor neuron syndrome

(2009) Alberto Esquenazi et al.   TOXICON

Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions

(2008) James Shorter et al.   EMBO JOURNAL

The Deubiquitinating Enzyme Ataxin-3, a Polyglutamine Disease Protein, Edits Lys63Linkages in Mixed Linkage Ubiquitin Chains

(2008) Brett J. Winborn et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp104 antagonizes α-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease

(2008) Christophe Lo Bianco et al.   JOURNAL OF CLINICAL INVESTIGATION

Induction of the Phase II Detoxification Pathway Suppresses Neuron Loss in Drosophila Models of Parkinson's Disease

(2008) K. Trinh et al.   JOURNAL OF NEUROSCIENCE

Substrate threading through the central pore of the Hsp104 chaperone as a common mechanism for protein disaggregation and prion propagation

(2008) Peter Tessarz et al.   MOLECULAR MICROBIOLOGY

Safety and Efficacy of Gene Transfer for Leber's Congenital Amaurosis

(2008) Albert M. Maguire et al.   NEW ENGLAND JOURNAL OF MEDICINE

Effect of Gene Therapy on Visual Function in Leber's Congenital Amaurosis

(2008) James W.B. Bainbridge et al.   NEW ENGLAND JOURNAL OF MEDICINE

Chaperone-dependent amyloid assembly protects cells from prion toxicity

(2008) P. M. Douglas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA