Legal but lethal: functional protein aggregation at the verge of toxicity

Modelling FUSopathies: focus on protein aggregation: Figure 1

(2015) Tatyana A. Shelkovnikova   BIOCHEMICAL SOCIETY TRANSACTIONS

Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis

(2014) Jean D. Sipe et al.   AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS

Significant Structural Differences between Transient Amyloid-β Oligomers and Less-Toxic Fibrils in Regions Known To Harbor Familial Alzheimer′s Mutations

(2014) Bidyut Sarkar et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Demystifying Heparan Sulfate–Protein Interactions

(2014) Ding Xu et al.   Annual Review of Biochemistry

Defined α-synuclein prion-like molecular assemblies spreading in cell culture

(2014) Suzana Aulić et al.   BMC NEUROSCIENCE

Biogenesis and secretion of exosomes

(2014) Joanna Kowal et al.   CURRENT OPINION IN CELL BIOLOGY

Heparin binding confers prion stability and impairs its aggregation

(2014) Tuane C. R. G. Vieira et al.   FASEB JOURNAL

Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms

(2014) Tatyana A. Shelkovnikova et al.   HUMAN MOLECULAR GENETICS

Parkinson's disease-linked human PARK9/ATP13A2 maintains zinc homeostasis and promotes α-Synuclein externalization via exosomes

(2014) Stephanie M.Y. Kong et al.   HUMAN MOLECULAR GENETICS

Architecture of Polyglutamine-containing Fibrils from Time-resolved Fluorescence Decay

(2014) Christoph Röthlein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

(2014) E. L. Scotter et al.   JOURNAL OF CELL SCIENCE

Functional Role of Tia1/Pub1 and Sup35 Prion Domains: Directing Protein Synthesis Machinery to the Tubulin Cytoskeleton

(2014) Xiang Li et al.   MOLECULAR CELL

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

Addition of exogenous α-synuclein preformed fibrils to primary neuronal cultures to seed recruitment of endogenous α-synuclein to Lewy body and Lewy neurite–like aggregates

(2014) Laura A Volpicelli-Daley et al.   Nature Protocols

Distinct Tau Prion Strains Propagate in Cells and Mice and Define Different Tauopathies

(2014) David W. Sanders et al.   NEURON

PASTA 2.0: an improved server for protein aggregation prediction

(2014) Ian Walsh et al.   NUCLEIC ACIDS RESEARCH

Mutant Human FUS Is Ubiquitously Mislocalized and Generates Persistent Stress Granules in Primary Cultured Transgenic Zebrafish Cells

(2014) Jamie Rae Acosta et al.   PLoS One

Self-assembled FUS binds active chromatin and regulates gene transcription

(2014) Liuqing Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Distinct synthetic A  prion strains producing different amyloid deposits in bigenic mice

(2014) J. Stohr et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

(2014) Leslie I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Serial propagation of distinct strains of A  prions from Alzheimer's disease patients

(2014) J. C. Watts et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Proteostasis impairment in protein-misfolding and -aggregation diseases

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

1st International Symposium on Stress-Associated RNA Granules in Human Disease and Viral Infection

(2014) Bruce Banfield et al.   Viruses-Basel

Full-length TDP-43 forms toxic amyloid oligomers that are present in frontotemporal lobar dementia-TDP patients

(2014) Yu-Sheng Fang et al.   Nature Communications

Prion propagation can occur in a prokaryote and requires the ClpB chaperone

(2014) Andy H Yuan et al.   eLife

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

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

Energy Failure

(2013) Divya Pathak et al.   ANNALS OF NEUROLOGY

Conformational Features of Tau Fibrils from Alzheimer’s Disease Brain Are Faithfully Propagated by Unmodified Recombinant Protein

(2013) Olga A. Morozova et al.   BIOCHEMISTRY

Boosting chaperone-mediated autophagy in vivo mitigates α-synuclein-induced neurodegeneration

(2013) Maria Xilouri et al.   BRAIN

Phosphorylation-Regulated Binding of RNA Polymerase II to Fibrous Polymers of Low-Complexity Domains

(2013) Ilmin Kwon et al.   CELL

Dual Specificity Kinase DYRK3 Couples Stress Granule Condensation/Dissolution to mTORC1 Signaling

(2013) Frank Wippich et al.   CELL

Distinct α-Synuclein Strains Differentially Promote Tau Inclusions in Neurons

(2013) Jing L. Guo et al.   CELL

Cytosolic proteins lose solubility as amyloid deposits in a transgenic mouse model of Alzheimer-type amyloidosis

(2013) Guilian Xu et al.   HUMAN MOLECULAR GENETICS

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

Intranuclear Aggregation of Mutant FUS/TLS as a Molecular Pathomechanism of Amyotrophic Lateral Sclerosis

(2013) Takao Nomura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lewy Body-like α-Synuclein Aggregates Resist Degradation and Impair Macroautophagy

(2013) Selcuk A. Tanik et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp90 Inhibits α-Synuclein Aggregation by Interacting with Soluble Oligomers

(2013) Soumya Daturpalli et al.   JOURNAL OF MOLECULAR BIOLOGY

Amyotrophic lateral sclerosis: an update on recent genetic insights

(2013) Yohei Iguchi et al.   JOURNAL OF NEUROLOGY

Synthetic Tau Fibrils Mediate Transmission of Neurofibrillary Tangles in a Transgenic Mouse Model of Alzheimer's-Like Tauopathy

(2013) M. Iba et al.   JOURNAL OF NEUROSCIENCE

Critical residues in the PMEL/Pmel17 N-terminus direct the hierarchical assembly of melanosomal fibrils

(2013) Ralf M. Leonhardt et al.   MOLECULAR BIOLOGY OF THE CELL

Neurodegeneration-Associated Protein Fragments as Short-Lived Substrates of the N-End Rule Pathway

(2013) Christopher S. Brower et al.   MOLECULAR CELL

Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Characterization of prion-like conformational changes of the neuronal isoform of Aplysia CPEB

(2013) Bindu L Raveendra et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Synthetic polyubiquitinated  -Synuclein reveals important insights into the roles of the ubiquitin chain in regulating its pathophysiology

(2013) M. Haj-Yahya et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cancer cell exosomes depend on cell-surface heparan sulfate proteoglycans for their internalization and functional activity

(2013) H. C. Christianson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Heparan sulfate proteoglycans mediate internalization and propagation of specific proteopathic seeds

(2013) B. B. Holmes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stress granules and cell signaling: more than just a passing phase?

(2013) Nancy Kedersha et al.   TRENDS IN BIOCHEMICAL SCIENCES

Structural and functional characterization of two alpha-synuclein strains

(2013) Luc Bousset et al.   Nature Communications

RNA Seeds Higher-Order Assembly of FUS Protein

(2013) Jacob C. Schwartz et al.   Cell Reports

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

(2013) Takashi Nonaka et al.   Cell Reports

Interactome of Two Diverse RNA Granules Links mRNA Localization to Translational Repression in Neurons

(2013) Renate Fritzsche et al.   Cell Reports

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

ATP13A2 (PARK9) protein levels are reduced in brain tissue of cases with Lewy bodies

(2013) Karen E Murphy et al.   Acta Neuropathologica Communications

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

(2012) Masato Kato et al.   CELL

Getting RNA and Protein in Phase

(2012) Stephanie C. Weber et al.   CELL

Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies

(2012) Tina W. Han et al.   CELL

The Amyloid State of Proteins in Human Diseases

(2012) David Eisenberg et al.   CELL

Nuclear bodies: multifunctional companions of the genome

(2012) Miroslav Dundr   CURRENT OPINION IN CELL BIOLOGY

FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2

(2012) J. C. Schwartz et al.   GENES & DEVELOPMENT

Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis

(2012) Yoshitaka Tashiro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cdc37/Hsp90 Protein Complex Disruption Triggers an Autophagic Clearance Cascade for TDP-43 Protein

(2012) Umesh K. Jinwal et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Requirements for Stress Granule Recruitment of Fused in Sarcoma (FUS) and TAR DNA-binding Protein of 43 kDa (TDP-43)

(2012) Eva Bentmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Contrasting Pathology of the Stress Granule Proteins TIA-1 and G3BP in Tauopathies

(2012) T. Vanderweyde et al.   JOURNAL OF NEUROSCIENCE

Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP

(2012) Anaïs Aulas et al.   Molecular Neurodegeneration

Exosomal cell-to-cell transmission of alpha synuclein oligomers

(2012) Karin M Danzer et al.   Molecular Neurodegeneration

Prions are a common mechanism for phenotypic inheritance in wild yeasts

(2012) Randal Halfmann et al.   NATURE

Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates

(2012) Sarah J. Parker et al.   NEUROCHEMISTRY INTERNATIONAL

The E. coli CsgB nucleator of curli assembles to  -sheet oligomers that alter the CsgA fibrillization mechanism

(2012) Q. Shu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pathological  -Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice

(2012) K. C. Luk et al.   SCIENCE

Cellular Strategies for Regulating Functional and Nonfunctional Protein Aggregation

(2012) Jörg Gsponer et al.   Cell Reports

FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA

(2012) Tomohiro Yamazaki et al.   Cell Reports

SERF Protein Is a Direct Modifier of Amyloid Fiber Assembly

(2012) S. Fabio Falsone et al.   Cell Reports

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

(2011) Heidi Olzscha et al.   CELL

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

(2011) Karli K. McDonald et al.   HUMAN MOLECULAR 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

Exosome-associated Tau Is Secreted in Tauopathy Models and Is Selectively Phosphorylated in Cerebrospinal Fluid in Early Alzheimer Disease

(2011) Sudad Saman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Selective transfer of exosomes from oligodendrocytes to microglia by macropinocytosis

(2011) D. Fitzner et al.   JOURNAL OF CELL SCIENCE

Cross-functional E3 ligases Parkin and C-terminus Hsp70-interacting protein in neurodegenerative disorders

(2011) Pravir Kumar et al.   JOURNAL OF NEUROCHEMISTRY

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

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

Distinct Roles In Vivo for the Ubiquitin-Proteasome System and the Autophagy-Lysosomal Pathway in the Degradation of  -Synuclein

(2011) D. Ebrahimi-Fakhari et al.   JOURNAL OF NEUROSCIENCE

A Diversity of Assembly Mechanisms of a Generic Amyloid Fold

(2011) Timo Eichner et al.   MOLECULAR CELL

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

(2011) Weirui Guo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Lysosomal dysfunction increases exosome-mediated alpha-synuclein release and transmission

(2011) Lydia Alvarez-Erviti et al.   NEUROBIOLOGY OF DISEASE

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

(2011) Smita Saxena et al.   NEURON

A yeast functional screen predicts new candidate ALS disease genes

(2011) J. Couthouis et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

(2011) Christian Münch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Signal transduction in neurons: effects of cellular prion protein on fyn kinase and ERK1/2 kinase

(2011) Vittorio Tomasi   Immunity & Ageing

Amyloid-β Peptide Is a Substrate of the Human 20S Proteasome

(2010) Xiaobei Zhao et al.   ACS Chemical Neuroscience

Identification of MOAG-4/SERF as a Regulator of Age-Related Proteotoxicity

(2010) Tjakko J. van Ham et al.   CELL

RNA granules: The good, the bad and the ugly

(2010) María Gabriela Thomas et al.   CELLULAR SIGNALLING

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

(2010) Daryl A. Bosco et al.   HUMAN MOLECULAR GENETICS

A Role for Huntington Disease Protein in Dendritic RNA Granules

(2010) Jeffrey N. Savas et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp70 and Hsp40 Functionally Interact with Soluble Mutant Huntingtin Oligomers in a Classic ATP-dependent Reaction Cycle

(2010) Gregor P. Lotz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Stable α-Synuclein Oligomers Strongly Inhibit Chaperone Activity of the Hsp70 System by Weak Interactions with J-domain Co-chaperones

(2010) Marie-Pierre Hinault et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Proteasome inhibition modeling nigral neuron degeneration in Parkinson’s disease

(2010) Wenjie Xie et al.   JOURNAL OF NEUROCHEMISTRY

Cell-Produced  -Synuclein Is Secreted in a Calcium-Dependent Manner by Exosomes and Impacts Neuronal Survival

(2010) E. Emmanouilidou et al.   JOURNAL OF NEUROSCIENCE

Induction of Amyloid Fibrils by the C-Terminal Fragments of TDP-43 in Amyotrophic Lateral Sclerosis

(2010) Allan K.-H. Chen et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

TIA1 Prevents Skipping of a Critical Exon Associated with Spinal Muscular Atrophy

(2010) N. N. Singh et al.   MOLECULAR AND CELLULAR BIOLOGY

Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments

(2010) Yong-Jie Zhang et al.   Molecular Neurodegeneration

Glycosaminoglycan Sulphation Affects the Seeded Misfolding of a Mutant Prion Protein

(2010) Victoria A. Lawson et al.   PLoS One

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

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

(2009) Simon Alberti et al.   CELL

RhoA/ROCK1 signaling regulates stress granule formation and apoptosis

(2009) Nien-Pei Tsai et al.   CELLULAR SIGNALLING

Chaperone proteostasis in Parkinson's disease: stabilization of the Hsp70/α-synuclein complex by Hip

(2009) Cintia Roodveldt et al.   EMBO JOURNAL

Tau fragmentation, aggregation and clearance: the dual role of lysosomal processing

(2009) Yipeng Wang et al.   HUMAN MOLECULAR GENETICS

The Molecular Chaperone Hsp90 Modulates Intermediate Steps of Amyloid Assembly of the Parkinson-related Protein α-Synuclein

(2009) S. Fabio Falsone et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

TDP-43 is recruited to stress granules in conditions of oxidative insult

(2009) Claudia Colombrita et al.   JOURNAL OF NEUROCHEMISTRY

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

Cross-Seeding Fibrillation of Q/N-Rich Proteins Offers New Pathomechanism of Polyglutamine Diseases

(2009) Y. Furukawa et al.   JOURNAL OF NEUROSCIENCE

Compositional Determinants of Prion Formation in Yeast

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

Hsp90 Regulates the Function of Argonaute 2 and Its Recruitment to Stress Granules and P-Bodies

(2009) Justin M. Pare et al.   MOLECULAR BIOLOGY OF THE CELL

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

(2009) Pei-Hsien Ren et al.   NATURE CELL BIOLOGY

Functional Amyloids As Natural Storage of Peptide Hormones in Pituitary Secretory Granules

(2009) S. K. Maji et al.   SCIENCE

Formation of stress granules inhibits apoptosis by suppressing stress-responsive MAPK pathways

(2008) Kyoko Arimoto et al.   NATURE CELL BIOLOGY

Stress granules: the Tao of RNA triage

(2008) Paul Anderson et al.   TRENDS IN BIOCHEMICAL SCIENCES

Enrichment of prion protein in exosomes derived from ovine cerebral spinal fluid

(2008) Laura J. Vella et al.   VETERINARY IMMUNOLOGY AND IMMUNOPATHOLOGY