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

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

Genetic interaction of hnRNPA2B1 and DNAJB6 in aDrosophilamodel of multisystem proteinopathy

(2016) Songqing Li et al.   HUMAN MOLECULAR GENETICS

Distinct Prion Domain Sequences Ensure Efficient Amyloid Propagation by Promoting Chaperone Binding or Processing In Vivo

(2016) Christine R. Langlois et al.   PLoS Genetics

Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1

(2015) Sami J. Barmada et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Differential effects of Ydj1 and Sis1 on Hsp70-mediated clearance of stress granules inSaccharomyces cerevisiae

(2015) Robert W. Walters et al.   RNA

Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis

(2014) K L Jackson et al.   GENE THERAPY

Myopathy-causing Mutations in an HSP40 Chaperone Disrupt Processing of Specific Client Conformers

(2014) Kevin C. Stein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion-promoted phosphorylation of heterologous amyloid is coupled with ubiquitin-proteasome system inhibition and toxicity

(2014) Zi Yang et al.   MOLECULAR MICROBIOLOGY

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

(2014) Sami J Barmada et al.   Nature Chemical Biology

Functional Diversification of Hsp40: Distinct J-Protein Functional Requirements for Two Prions Allow for Chaperone-Dependent Prion Selection

(2014) Julia M. Harris et al.   PLoS Genetics

Heritable Remodeling of Yeast Multicellularity by an Environmentally Responsive Prion

(2013) Daniel L. Holmes et al.   CELL

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

(2013) Sae-Hun Park et al.   CELL

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

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders

(2013) J. Janssens 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

Stress granules as crucibles of ALS pathogenesis

(2013) Yun R. Li et al.   JOURNAL OF CELL BIOLOGY

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models

(2013) Hyung-Jun Kim et al.   NATURE GENETICS

The Type II Hsp40 Sis1 Cooperates with Hsp70 and the E3 Ligase Ubr1 to Promote Degradation of Terminally Misfolded Cytosolic Protein

(2013) Daniel W. Summers et al.   PLoS One

Improved Blue, Green, and Red Fluorescent Protein Tagging Vectors for S. cerevisiae

(2013) Sidae Lee et al.   PLoS One

Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

(2013) Takashi Nonaka et al.   Cell Reports

Heterologous Gln/Asn-Rich Proteins Impede the Propagation of Yeast Prions by Altering Chaperone Availability

(2013) Zi Yang et al.   PLoS Genetics

TDP-43 skeins show properties of amyloid in a subset of ALS cases

(2012) John L. Robinson et al.   ACTA NEUROPATHOLOGICA

Prions in Yeast

(2012) S. W. Liebman et al.   GENETICS

Molecular chaperones and stress-inducible protein-sorting factors coordinate the spatiotemporal distribution of protein aggregates

(2012) Liliana Malinovska et al.   MOLECULAR BIOLOGY OF THE CELL

A yeast model of optineurin proteinopathy reveals a unique aggregation pattern associated with cellular toxicity

(2012) Dmitry Kryndushkin et al.   MOLECULAR MICROBIOLOGY

Regulated protein aggregation: stress granules and neurodegeneration

(2012) Benjamin Wolozin   Molecular Neurodegeneration

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

(2012) Maria Armakola et al.   NATURE GENETICS

A Yeast Prion, Mod5, Promotes Acquired Drug Resistance and Cell Survival Under Environmental Stress

(2012) G. Suzuki et al.   SCIENCE

Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy

(2011) Matthew B. Harms et al.   ANNALS OF NEUROLOGY

Functions of Yeast Hsp40 Chaperone Sis1p Dispensable for Prion Propagation but Important for Prion Curing and Protection From Prion Toxicity

(2011) P. Aaron Kirkland et al.   GENETICS

A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions

(2011) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp42 is required for sequestration of protein aggregates into deposition sites inSaccharomyces cerevisiae

(2011) Sebastian Specht et al.   JOURNAL OF CELL BIOLOGY

RNA-binding proteins with prion-like domains in ALS and FTLD-U

(2011) Aaron D. Gitler et al.   Prion

Pathogenic TARDBP Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: Disease-Associated Pathways

(2011) Sami J. Barmada et al.   REVIEWS IN THE NEUROSCIENCES

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

[SWI+], the Prion Formed by the Chromatin Remodeling Factor Swi1, Is Highly Sensitive to Alterations in Hsp70 Chaperone System Activity

(2011) Justin K. Hines et al.   PLoS Genetics

FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis

(2011) Dmitry Kryndushkin et al.   Protein & Cell

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

(2010) C. Lagier-Tourenne et al.   HUMAN MOLECULAR GENETICS

Interaction with Polyglutamine Aggregates Reveals a Q/N-rich Domain in TDP-43

(2010) Rodrigo A. Fuentealba et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Seeded Aggregation and Toxicity of α-Synuclein and Tau

(2010) Takashi Nonaka et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

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

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

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

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

Ssa1 Overexpression and [PIN+] Variants Cure [PSI+] by Dilution of Aggregates

(2009) Vidhu Mathur et al.   JOURNAL OF MOLECULAR BIOLOGY

Global Analysis of TDP-43 Interacting Proteins Reveals Strong Association with RNA Splicing and Translation Machinery

(2009) Brian D. Freibaum et al.   JOURNAL OF PROTEOME RESEARCH

Compositional Determinants of Prion Formation in Yeast

(2009) J. A. Toombs et al.   MOLECULAR AND CELLULAR BIOLOGY

Reciprocal Efficiency of RNQ1 and Polyglutamine Detoxification in the Cytosol and Nucleus

(2009) Peter M. Douglas et al.   MOLECULAR BIOLOGY OF THE CELL

Transmission and spreading of tauopathy in transgenic mouse brain

(2009) Florence Clavaguera et al.   NATURE CELL BIOLOGY

The yeast global transcriptional co-repressor protein Cyc8 can propagate as a prion

(2009) Basant K. Patel et al.   NATURE CELL BIOLOGY

Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

(2008) Lionel M. Igaz et al.   AMERICAN JOURNAL OF PATHOLOGY

Functionally Redundant Isoforms of a Yeast Hsp70 Chaperone Subfamily Have Different Antiprion Effects

(2008) D. Sharma et al.   GENETICS

A Regulatory Role of the Rnq1 Nonprion Domain for Prion Propagation and Polyglutamine Aggregates

(2008) H. Kurahashi et al.   MOLECULAR AND CELLULAR BIOLOGY

Variant-specific [PSI+] Infection Is Transmitted by Sup35 Polymers within [PSI+] Aggregates with Heterogeneous Protein Composition

(2008) Sviatoslav N. Bagriantsev et al.   MOLECULAR BIOLOGY OF THE CELL

In Vivo Monitoring of the Prion Replication Cycle Reveals a Critical Role for Sis1 in Delivering Substrates to Hsp104

(2008) Kimberly A. Tipton et al.   MOLECULAR CELL

Newly identified prion linked to the chromatin-remodeling factor Swi1 in Saccharomyces cerevisiae

(2008) Zhiqiang Du et al.   NATURE GENETICS

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Specificity of the J-protein Sis1 in the propagation of 3 yeast prions

(2008) T. Higurashi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

(2008) B. S. Johnson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

(2008) Nicola J. Rutherford et al.   PLoS Genetics