Prion-like propagation as a pathogenic principle in frontotemporal dementia

Axonal transport and secretion of fibrillar forms of α-synuclein, Aβ42 peptide and HTTExon 1

(2016) Michel Brahic et al.   ACTA NEUROPATHOLOGICA

Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils

(2016) Shotaro Shimonaka et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Short Fibrils Constitute the Major Species of Seed-Competent Tau in the Brains of Mice Transgenic for Human P301S Tau

(2016) S. J. Jackson et al.   JOURNAL OF NEUROSCIENCE

A critical appraisal of the pathogenic protein spread hypothesis of neurodegeneration

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

Prions and Protein Assemblies that Convey Biological Information in Health and Disease

(2016) David W. Sanders et al.   NEURON

Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS

(2015) Yazi D. Ke et al.   ACTA NEUROPATHOLOGICA

Propagation of Aß pathology: hypotheses, discoveries, and yet unresolved questions from experimental and human brain studies

(2015) Yvonne S. Eisele et al.   ACTA NEUROPATHOLOGICA

Propagation of Aβ, tau and α-synuclein pathology between experimental models and human reality: prions, propagons and propaganda

(2015) Toshiki Uchihara et al.   ACTA NEUROPATHOLOGICA

Biochemical classification of tauopathies by immunoblot, protein sequence and mass spectrometric analyses of sarkosyl-insoluble and trypsin-resistant tau

(2015) Sayuri Taniguchi-Watanabe et al.   ACTA NEUROPATHOLOGICA

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

(2015) Adam K. Walker et al.   ACTA NEUROPATHOLOGICA

Deep clinical and neuropathological phenotyping of Pick disease

(2015) David J. Irwin et al.   ANNALS OF NEUROLOGY

Neurodegenerative Diseases: Expanding the Prion Concept

(2015) Lary C. Walker et al.   Annual Review of Neuroscience

4-Hydroxynonenal induces persistent insolubilization of TDP-43 and alters its intracellular localization

(2015) Chihana Kabuta et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

Propagation of Pathology through Brain Networks in Neurodegenerative Diseases: From Molecules to Clinical Phenotypes

(2015) Federica Agosta et al.   CNS Neuroscience & Therapeutics

Removing endogenous tau does not prevent tau propagation yet reduces its neurotoxicity

(2015) S. Wegmann et al.   EMBO JOURNAL

Tau Trimers Are the Minimal Propagation Unit Spontaneously Internalized to Seed Intracellular Aggregation

(2015) Hilda Mirbaha et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Distinct Therapeutic Mechanisms of Tau Antibodies

(2015) Kristen E. Funk et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 is intercellularly transmitted across axon terminals

(2015) Marisa S. Feiler et al.   JOURNAL OF CELL BIOLOGY

Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase II

(2015) Kathleen A. Burke et al.   MOLECULAR CELL

Depletion of microglia and inhibition of exosome synthesis halt tau propagation

(2015) Hirohide Asai et al.   NATURE NEUROSCIENCE

Spreading of pathology in neurodegenerative diseases: a focus on human studies

(2015) Johannes Brettschneider et al.   NATURE REVIEWS NEUROSCIENCE

Tau in physiology and pathology

(2015) Yipeng Wang et al.   NATURE REVIEWS NEUROSCIENCE

Intracerebral injection of preformed synthetic tau fibrils initiates widespread tauopathy and neuronal loss in the brains of tau transgenic mice

(2015) Eve Peeraer et al.   NEUROBIOLOGY OF DISEASE

Tau Immunotherapy

(2015) Einar M. Sigurdsson   Neurodegenerative Diseases

Review: Prion-like mechanisms of transactive response DNA binding protein of 43 kDa (TDP-43) in amyotrophic lateral sclerosis (ALS)

(2015) Phillip Smethurst et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Review: An update on clinical, genetic and pathological aspects of frontotemporal lobar degenerations

(2015) Tammaryn Lashley et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Invited review: Frontotemporal dementia caused bymicrotubule-associated protein taugene (MAPT) mutations: a chameleon for neuropathology and neuroimaging

(2015) Bernardino Ghetti et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Invited review: Prion-like transmission and spreading of tau pathology

(2015) Florence Clavaguera et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

From nucleation to widespread propagation: A prion-like concept for ALS

(2015) Zuzanna Maniecka et al.   VIRUS RESEARCH

An acetylation switch controls TDP-43 function and aggregation propensity

(2015) Todd J. Cohen et al.   Nature Communications

Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain

(2015) Margaret M. P. Pearce et al.   Nature Communications

Neuronal uptake and propagation of a rare phosphorylated high-molecular-weight tau derived from Alzheimer’s disease brain

(2015) Shuko Takeda et al.   Nature Communications

Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure

(2015) Xuebing Ding et al.   Oncotarget

Synaptic Contacts Enhance Cell-to-Cell Tau Pathology Propagation

(2015) Sara Calafate et al.   Cell Reports

A novel in vivo model of tau propagation with rapid and progressive neurofibrillary tangle pathology: the pattern of spread is determined by connectivity, not proximity

(2014) Zeshan Ahmed et al.   ACTA NEUROPATHOLOGICA

Differential induction and spread of tau pathology in young PS19 tau transgenic mice following intracerebral injections of pathological tau from Alzheimer’s disease or corticobasal degeneration brains

(2014) Susana Boluda et al.   ACTA NEUROPATHOLOGICA

Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD)

(2014) Johannes Brettschneider et al.   ACTA NEUROPATHOLOGICA

Frontotemporal dementia: a bridge between dementia and neuromuscular disease

(2014) Adeline S.L. Ng et al.   Annals of the New York Academy of Sciences

Focality, stochasticity and neuroanatomic propagation in ALS pathogenesis

(2014) John Ravits   EXPERIMENTAL NEUROLOGY

Extracellular Monomeric Tau Protein Is Sufficient to Initiate the Spread of Tau Protein Pathology

(2014) Claire H. Michel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Neuronal activity regulates extracellular tau in vivo

(2014) Kaoru Yamada et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Global analysis of protein structural changes in complex proteomes

(2014) Yuehan Feng et al.   NATURE BIOTECHNOLOGY

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

(2014) Jing L Guo et al.   NATURE MEDICINE

Transneuronal propagation of mutant huntingtin contributes to non–cell autonomous pathology in neurons

(2014) Eline Pecho-Vrieseling et al.   NATURE NEUROSCIENCE

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

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

(2014) David W. Sanders et al.   NEURON

TDP-43 N terminus encodes a novel ubiquitin-like fold and its unfolded form in equilibrium that can be shifted by binding to ssDNA

(2014) Haina Qin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

(2014) S. Mizielinska et al.   SCIENCE

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

Limited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases

(2014) Emily Feneberg et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathies

(2014) Simon Dujardin et al.   Acta Neuropathologica Communications

Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins

(2013) Kohji Mori et al.   ACTA NEUROPATHOLOGICA

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

(2013) Johannes Brettschneider et al.   ANNALS OF NEUROLOGY

“Prion-Like” Templated Misfolding in Tauopathies

(2013) Florence Clavaguera et al.   BRAIN PATHOLOGY

The dendritic hypothesis for Alzheimer's disease pathophysiology

(2013) J. Nicholas Cochran et al.   BRAIN RESEARCH BULLETIN

Distinct α-Synuclein Strains Differentially Promote Tau Inclusions in Neurons

(2013) Jing L. Guo et al.   CELL

Physiological release of endogenous tau is stimulated by neuronal activity

(2013) Amy M Pooler et al.   EMBO REPORTS

Seeded strain-like transmission of β-amyloid morphotypes in APP transgenic mice

(2013) Götz Heilbronner et al.   EMBO REPORTS

Longitudinal white matter changes in frontotemporal dementia subtypes

(2013) Bonnie Y. K. Lam et al.   HUMAN BRAIN MAPPING

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation

(2013) Yong-Jie Zhang 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

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

Tau pathology and neurodegeneration

(2013) Maria Grazia Spillantini et al.   LANCET NEUROLOGY

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases

(2013) Mathias Jucker et al.   NATURE

Amyotrophic lateral sclerosis—a model of corticofugal axonal spread

(2013) Heiko Braak et al.   Nature Reviews Neurology

Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43

(2013) Peter J Lukavsky et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Anti-Tau Antibodies that Block Tau Aggregate Seeding In Vitro Markedly Decrease Pathology and Improve Cognition In Vivo

(2013) Kiran Yanamandra et al.   NEURON

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Predominance of spliceosomal complex formation over polyadenylation site selection in TDP-43 autoregulation

(2013) S. Bembich et al.   NUCLEIC ACIDS RESEARCH

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

Propagation of tau pathology in Alzheimer’s disease: identification of novel therapeutic targets

(2013) Amy M Pooler et al.   Alzheimers Research & Therapy

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

ALS-Associated FUS Mutations Result in Compromised FUS Alternative Splicing and Autoregulation

(2013) Yueqin Zhou et al.   PLoS Genetics

Quaternary Structure of Pathological Prion Protein as a Determining Factor of Strain-Specific Prion Replication Dynamics

(2013) Florent Laferrière et al.   PLoS Pathogens

Molecular analysis and biochemical classification of TDP-43 proteinopathy

(2012) Hiroshi Tsuji et al.   BRAIN

The spectrum of disease in chronic traumatic encephalopathy

(2012) Ann C. McKee et al.   BRAIN

Misregulated RNA processing in amyotrophic lateral sclerosis

(2012) Magdalini Polymenidou et al.   BRAIN RESEARCH

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

(2012) Masato Kato 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

Functional network connectivity in the behavioral variant of frontotemporal dementia

(2012) Massimo Filippi et al.   CORTEX

Loss of fused in sarcoma (FUS) promotes pathological Tau splicing

(2012) Denise Orozco et al.   EMBO REPORTS

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

Extracellular Tau Levels Are Influenced by Variability in Tau That Is Associated with Tauopathies

(2012) Celeste M. Karch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small Misfolded Tau Species Are Internalized via Bulk Endocytosis and Anterogradely and Retrogradely Transported in Neurons

(2012) Jessica W. Wu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Trans-cellular Propagation of Tau Aggregation by Fibrillar Species

(2012) Najla Kfoury et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion-like spread of protein aggregates in neurodegeneration: Figure 1.

(2012) Magdalini Polymenidou et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Spreading of Neurodegenerative Pathology via Neuron-to-Neuron Transmission of  -Amyloid

(2012) S. Nath et al.   JOURNAL OF NEUROSCIENCE

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

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

β-Sheet Core of Tau Paired Helical Filaments Revealed by Solid-State NMR

(2012) Venita Daebel et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices

(2012) Cadman Leggett et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

(2012) Elisa Majounie et al.   LANCET NEUROLOGY

Regulated protein aggregation: stress granules and neurodegeneration

(2012) Benjamin Wolozin   Molecular Neurodegeneration

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Constitutive secretion of tau protein by an unconventional mechanism

(2012) Xiyun Chai et al.   NEUROBIOLOGY OF DISEASE

A Network Diffusion Model of Disease Progression in Dementia

(2012) Ashish Raj et al.   NEURON

Predicting Regional Neurodegeneration from the Healthy Brain Functional Connectome

(2012) Juan Zhou et al.   NEURON

Propagation of Tau Pathology in a Model of Early Alzheimer's Disease

(2012) Alix de Calignon et al.   NEURON

Trans-Synaptic Spread of Tau Pathology In Vivo

(2012) Li Liu et al.   PLoS One

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

(2012) K. C. Luk et al.   SCIENCE

Alzheimer brain-derived tau oligomers propagate pathology from endogenous tau

(2012) Cristian A. Lasagna-Reeves et al.   Scientific Reports

Mechanisms of Protein Seeding in Neurodegenerative Diseases

(2012) Lary C. Walker et al.   JAMA Neurology

A harmonized classification system for FTLD-TDP pathology

(2011) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations

(2011) Manuela Neumann et al.   BRAIN

Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia

(2011) Katya Rascovsky et al.   BRAIN

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

White Matter Damage in Frontotemporal Lobar Degeneration Spectrum

(2011) F. Agosta et al.   CEREBRAL CORTEX

Proteostasis of tau. Tau overexpression results in its secretion via membrane vesicles

(2011) Diana Simón et al.   FEBS LETTERS

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

Passive Immunization with Anti-Tau Antibodies in Two Transgenic Models

(2011) Xiyun Chai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Seeding of Normal Tau by Pathological Tau Conformers Drives Pathogenesis of Alzheimer-like Tangles

(2011) Jing L. Guo 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

A “Two-hit” Hypothesis for Inclusion Formation by Carboxyl-terminal Fragments of TDP-43 Protein Linked to RNA Depletion and Impaired Microtubule-dependent Transport

(2011) G. Scott Pesiridis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular Dissection of TDP-43 Proteinopathies

(2011) Masato Hasegawa et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Pathological Staging of Frontotemporal Lobar Degeneration

(2011) Jillian J. Kril et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Passive immunization targeting pathological phospho-tau protein in a mouse model reduces functional decline and clears tau aggregates from the brain

(2011) Allal Boutajangout et al.   JOURNAL OF NEUROCHEMISTRY

In Vivo Microdialysis Reveals Age-Dependent Decrease of Brain Interstitial Fluid Tau Levels in P301S Human Tau Transgenic Mice

(2011) K. Yamada et al.   JOURNAL OF NEUROSCIENCE

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

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

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

Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS

(2011) D. Ito et al.   NEUROLOGY

Classification of primary progressive aphasia and its variants

(2011) M. L. Gorno-Tempini et al.   NEUROLOGY

Exogenous α-Synuclein Fibrils Induce Lewy Body Pathology Leading to Synaptic Dysfunction and Neuron Death

(2011) Laura A. Volpicelli-Daley et al.   NEURON

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

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

(2011) Aaron D. Gitler et al.   Prion

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

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

(2011) L. I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration

(2010) Hazel Urwin et al.   ACTA NEUROPATHOLOGICA

Divergent network connectivity changes in behavioural variant frontotemporal dementia and Alzheimer’s disease

(2010) Juan Zhou et al.   BRAIN

TDP-43 regulates its mRNA levels through a negative feedback loop

(2010) Youhna M Ayala et al.   EMBO JOURNAL

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

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

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor

(2010) C. M. Dewey et al.   MOLECULAR AND CELLULAR BIOLOGY

Prion-like transmission of protein aggregates in neurodegenerative diseases

(2010) Patrik Brundin et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Cell-to-cell transmission of non-prion protein aggregates

(2010) Seung-Jae Lee et al.   Nature Reviews Neurology

Clinical staging and disease progression in frontotemporal dementia

(2010) E. Mioshi et al.   NEUROLOGY

Alzheimer's Disease

(2010) Henry W. Querfurth et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

Non-ATG-initiated translation directed by microsatellite expansions

(2010) T. Zu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Generating a Prion with Bacterially Expressed Recombinant Prion Protein

(2010) F. Wang et al.   SCIENCE

The propagation of prion-like protein inclusions in neurodegenerative diseases

(2010) Michel Goedert et al.   TRENDS IN NEUROSCIENCES

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

FUS pathology in basophilic inclusion body disease

(2009) David G. Munoz et al.   ACTA NEUROPATHOLOGICA

Clinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies

(2009) Felix Geser et al.   ARCHIVES OF NEUROLOGY

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

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

(2009) Simon Alberti et al.   CELL

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

Propagation of Tau Misfolding from the Outside to the Inside of a Cell

(2009) Bess Frost 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

Compositional Determinants of Prion Formation in Yeast

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

Eukaryotic Stress Granules: The Ins and Outs of Translation

(2009) J. Ross Buchan et al.   MOLECULAR CELL

Beyond the prion principle

(2009) Adriano Aguzzi   NATURE

Transmission and spreading of tauopathy in transgenic mouse brain

(2009) Florence Clavaguera et al.   NATURE CELL BIOLOGY

RNA granules: post-transcriptional and epigenetic modulators of gene expression

(2009) Paul Anderson et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The heritability and genetics of frontotemporal lobar degeneration

(2009) J. D. Rohrer et al.   NEUROLOGY

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

(2009) J. M. Ravits et al.   NEUROLOGY

The Transcellular Spread of Cytosolic Amyloids, Prions, and Prionoids

(2009) Adriano Aguzzi et al.   NEURON

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

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

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Tau phosphorylation: the therapeutic challenge for neurodegenerative disease

(2009) Diane P. Hanger et al.   TRENDS IN MOLECULAR MEDICINE

Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis

(2008) Takashi Kasai et al.   ACTA NEUROPATHOLOGICA

Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease

(2008) Kengo Fujita et al.   ACTA NEUROPATHOLOGICA

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

The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response

(2008) Mattias K Andersson et al.   BMC CELL BIOLOGY

Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury

(2008) Katie Moisse et al.   BRAIN RESEARCH

Selective functional, regional, and neuronal vulnerability in frontotemporal dementia

(2008) William W Seeley   CURRENT OPINION IN NEUROLOGY

Variations in the progranulin gene affect global gene expression in frontotemporal lobar degeneration

(2008) Alice S. Chen-Plotkin et al.   HUMAN MOLECULAR GENETICS

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

(2008) I.-Fan Wang et al.   JOURNAL OF NEUROCHEMISTRY

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

(2008) Vivianna M Van Deerlin et al.   LANCET NEUROLOGY