Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis

The olfactory bulb as the entry site for prion-like propagation in neurodegenerative diseases

(2018) Nolwen L. Rey et al.   NEUROBIOLOGY OF DISEASE

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

Pathological Tau Strains from Human Brains Recapitulate the Diversity of Tauopathies in Nontransgenic Mouse Brain

(2017) Sneha Narasimhan et al.   JOURNAL OF NEUROSCIENCE

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

Injected Amyloid Beta in the Olfactory Bulb Transfers to Other Brain Regions via Neural Connections in Mice

(2017) Baixuan He et al.   MOLECULAR NEUROBIOLOGY

Silencing strategies for therapy of SOD1-mediated ALS

(2017) Brigitte van Zundert et al.   NEUROSCIENCE LETTERS

ATP as a biological hydrotrope

(2017) Avinash Patel et al.   SCIENCE

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

(2017) Tariq Afroz et al.   Nature Communications

Prion strains depend on different endocytic routes for productive infection

(2017) Andrea Fehlinger et al.   Scientific Reports

Anti-α-synuclein immunotherapy reduces α-synuclein propagation in the axon and degeneration in a combined viral vector and transgenic model of synucleinopathy

(2017) Brian Spencer et al.   Acta Neuropathologica Communications

Formation and spreading of TDP-43 aggregates in cultured neuronal and glial cells demonstrated by time-lapse imaging

(2017) Tomohiro Ishii et al.   PLoS One

α-synuclein transfer through tunneling nanotubes occurs in SH-SY5Y cells and primary brain pericytes from Parkinson’s disease patients

(2017) Birger Victor Dieriks et al.   Scientific Reports

Co-regulation of mRNA translation by TDP-43 and Fragile X Syndrome protein FMRP

(2016) Pritha Majumder et al.   ACTA NEUROPATHOLOGICA

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

(2016) Michel Brahic et al.   ACTA NEUROPATHOLOGICA

Exosome secretion is a key pathway for clearance of pathological TDP-43

(2016) Yohei Iguchi et al.   BRAIN

TBPH/TDP-43 modulates translation of Drosophila futsch mRNA through an UG-rich sequence within its 5′UTR

(2016) Maurizio Romano et al.   BRAIN RESEARCH

There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS

(2016) Aaron D. Gitler et al.   BRAIN RESEARCH

Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease

(2016) Zachary M. March et al.   BRAIN RESEARCH

ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure

(2016) Saumya Jain et al.   CELL

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Compositional Control of Phase-Separated Cellular Bodies

(2016) Salman F. Banani et al.   CELL

Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers

(2016) Yi Lin et al.   CELL

Identification of possible siRNA molecules for TDP43 mutants causing amyotrophic lateral sclerosis: In silico design and molecular dynamics study

(2016) Vishwambhar Vishnu Bhandare et al.   COMPUTATIONAL BIOLOGY AND CHEMISTRY

Therapeutic rAAVrh10 MediatedSOD1Silencing in AdultSOD1G93AMice and Nonhuman Primates

(2016) Florie Borel et al.   HUMAN GENE THERAPY

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

Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis–like disease

(2016) Elaheh Ekhtiari Bidhendi et al.   JOURNAL OF CLINICAL INVESTIGATION

Unique pathological tau conformers from Alzheimer’s brains transmit tau pathology in nontransgenic mice

(2016) Jing L. Guo et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Widespread transneuronal propagation of α-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson’s disease

(2016) Nolwen L. Rey et al.   JOURNAL OF EXPERIMENTAL MEDICINE

The antibody aducanumab reduces Aβ plaques in Alzheimer’s disease

(2016) Jeff Sevigny et al.   NATURE

In vitro prion-like behaviour of TDP-43 in ALS

(2016) Phillip Smethurst et al.   NEUROBIOLOGY OF DISEASE

TDP-43 pathology and cognition in ALS

(2016) Johannes Prudlo et al.   NEUROLOGY

Tau Prion Strains Dictate Patterns of Cell Pathology, Progression Rate, and Regional Vulnerability In Vivo

(2016) Sarah K. Kaufman et al.   NEURON

Tauopathies as clinicopathological entities

(2016) David J. Irwin   PARKINSONISM & RELATED DISORDERS

Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110

(2016) Maria Nagy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Luminidependens (LD) is an Arabidopsis protein with prion behavior

(2016) Sohini Chakrabortee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

RIPK1 mediates axonal degeneration by promoting inflammation and necroptosis in ALS

(2016) Yasushi Ito et al.   SCIENCE

FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons

(2016) Julia Higelin et al.   Frontiers in Cellular Neuroscience

Two mutations G335D and Q343R within the amyloidogenic core region of TDP-43 influence its aggregation and inclusion formation

(2016) Lei-Lei Jiang et al.   Scientific Reports

Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72 -ALS/FTD

(2016) Thomas Westergard et al.   Cell Reports

ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43

(2016) Liangzhong Lim et al.   PLOS BIOLOGY

Tunneling nanotube (TNT)-mediated neuron-to neuron transfer of pathological Tau protein assemblies

(2016) Meryem Tardivel et al.   Acta Neuropathologica Communications

Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity

(2016) Brittany N. Flores et al.   PLoS One

TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins

(2016) S. Prpar Mihevc et al.   Scientific Reports

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

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

Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways

(2015) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

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

Inverse Size Scaling of the Nucleolus by a Concentration-Dependent Phase Transition

(2015) Stephanie C. Weber et al.   CURRENT BIOLOGY

Prion-like domains in RNA binding proteins are essential for building subnuclear paraspeckles

(2015) Sven Hennig et al.   JOURNAL OF CELL BIOLOGY

TDP-43 is intercellularly transmitted across axon terminals

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

Emerging mechanisms of molecular pathology in ALS

(2015) Owen M. Peters et al.   JOURNAL OF CLINICAL INVESTIGATION

Amyotrophic lateral sclerosis: Is the spinal fluid pathway involved in seeding and spread?

(2015) Richard Smith et al.   MEDICAL HYPOTHESES

Autophagy receptor defects and ALS-FTLD

(2015) Veronika Majcher et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

It’s Raining Liquids: RNA Tunes Viscoelasticity and Dynamics of Membraneless Organelles

(2015) Lin Guo et al.   MOLECULAR CELL

Phase Transition of a Disordered Nuage Protein Generates Environmentally Responsive Membraneless Organelles

(2015) Timothy J. Nott et al.   MOLECULAR CELL

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

Depletion of microglia and inhibition of exosome synthesis halt tau propagation

(2015) Hirohide Asai et al.   NATURE NEUROSCIENCE

Stepwise acquirement of hallmark neuropathology in FUS-ALS iPSC models depends on mutation type and neuronal aging

(2015) Julia Japtok et al.   NEUROBIOLOGY OF DISEASE

Intrastriatal injection of pre-formed mouse α-synuclein fibrils into rats triggers α-synuclein pathology and bilateral nigrostriatal degeneration

(2015) Katrina L. Paumier et al.   NEUROBIOLOGY OF DISEASE

Role of Immunity and Inflammation in the Pathophysiology of Neurodegenerative Diseases

(2015) Marc Fakhoury   Neurodegenerative Diseases

Familial Amyotrophic Lateral Sclerosis

(2015) Kevin Boylan   NEUROLOGIC CLINICS

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

(2015) Tetsuro Murakami et al.   NEURON

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

(2015) Owen M. Peters et al.   NEURON

An autopsy case of familial amyotrophic lateral sclerosis with aTARDBPQ343R mutation

(2015) Koichi Okamoto et al.   NEUROPATHOLOGY

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

TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets

(2015) Emma L. Scotter et al.   Neurotherapeutics

Readers of poly(ADP-ribose): designed to be fit for purpose

(2015) Federico Teloni et al.   NUCLEIC ACIDS RESEARCH

Prion aggregates transfer through tunneling nanotubes in endocytic vesicles

(2015) Seng Zhu et al.   Prion

Structural and kinetic analysis of protein-aggregate strains in vivo using binary epitope mapping

(2015) Johan Bergh et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism

(2015) Stanley B. Prusiner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

Parkinson's disease as a member of Prion-like disorders

(2015) Maria Eugenia Herva et al.   VIRUS RESEARCH

Liquid demixing of intrinsically disordered proteins is seeded by poly(ADP-ribose)

(2015) Matthias Altmeyer et al.   Nature Communications

Human TDP-43 and FUS selectively affect motor neuron maturation and survival in a murine cell model of ALS by non-cell-autonomous mechanisms

(2015) Nicole Wächter et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Risk factors for amyotrophic lateral sclerosis

(2015) Fang Fang et al.   Clinical Epidemiology

Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways

(2015) Manaal Fatima et al.   Acta Neuropathologica Communications

Formal Comment on Schorr GS, Falcone EA, Moretti DJ, Andrews RD (2014) First Long-Term Behavioral Records from Cuvier’s Beaked Whales (Ziphius cavirostris) Reveal Record-Breaking Dives. PLoS ONE 9(3): e92633. doi:10.1371/journal.pone.0092633

(2015) Peter L. Tyack et al.   PLoS One

TDP-43 regulatesβ-adducin(Add2) transcript stability

(2015) Luisa Costessi et al.   RNA Biology

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

Direct evidence of Parkinson pathology spread from the gastrointestinal tract to the brain in rats

(2014) Staffan Holmqvist et al.   ACTA NEUROPATHOLOGICA

C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration

(2014) Stephanie May et al.   ACTA NEUROPATHOLOGICA

Experimental transmissibility of mutant SOD1 motor neuron disease

(2014) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

Intrinsically Disordered Proteins and Intrinsically Disordered Protein Regions

(2014) Christopher J. Oldfield et al.   Annual Review of Biochemistry

Liquid-Liquid Phase Separation in Biology

(2014) Anthony A. Hyman et al.   Annual Review of Cell and Developmental Biology

Alzheimer disease: Epidemiology, diagnostic criteria, risk factors and biomarkers

(2014) Christiane Reitz et al.   BIOCHEMICAL PHARMACOLOGY

Disease origin and progression in amyotrophic lateral sclerosis: an immunology perspective

(2014) Andrea Malaspina et al.   INTERNATIONAL IMMUNOLOGY

Mutagenic and cytotoxic potential of Endosulfan and Lambda-cyhalothrin — In vitro study describing individual and combined effects of pesticides

(2014) Umber Saleem et al.   JOURNAL OF ENVIRONMENTAL SCIENCES

Reducing C-Terminal-Truncated Alpha-Synuclein by Immunotherapy Attenuates Neurodegeneration and Propagation in Parkinson's Disease-Like Models

(2014) D. Games et al.   JOURNAL OF NEUROSCIENCE

Disturbed mitochondrial dynamics and neurodegenerative disorders

(2014) Florence Burté et al.   Nature Reviews Neurology

The role of FUS gene variants in neurodegenerative diseases

(2014) Hao Deng et al.   Nature Reviews Neurology

Spreading of amyloid-β peptides via neuritic cell-to-cell transfer is dependent on insufficient cellular clearance

(2014) Jakob Domert et al.   NEUROBIOLOGY OF DISEASE

Tar DNA-binding protein-43 (TDP-43) regulates axon growth in vitro and in vivo

(2014) Vineeta Bhasker Tripathi et al.   NEUROBIOLOGY OF DISEASE

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

(2014) Nael H. Alami et al.   NEURON

Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

(2014) Diane B. Re et al.   NEURON

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

(2014) David W. Sanders et al.   NEURON

Motor Neuron Death in ALS: Programmed by Astrocytes?

(2014) Sheila K. Pirooznia et al.   NEURON

Incidence and Geographical Variation of Amyotrophic Lateral Sclerosis (ALS) in Southern Germany – Completeness of the ALS Registry Swabia

(2014) Hatice Uenal et al.   PLoS One

Prion-like activity of Cu/Zn superoxide dismutase

(2014) Leslie I Grad et al.   Prion

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

The Structure of Human Prions: From Biology to Structural Models—Considerations and Pitfalls

(2014) Claudia Acevedo-Morantes et al.   Viruses-Basel

A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis

(2014) Mariko Ogawa et al.   Frontiers in Cellular Neuroscience

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

Alpha-synuclein biology in Lewy body diseases

(2014) Woojin Scott Kim et al.   Alzheimers Research & Therapy

α-Synuclein Immunotherapy Blocks Uptake and Templated Propagation of Misfolded α-Synuclein and Neurodegeneration

(2014) Hien T. Tran et al.   Cell Reports

Alpha-synuclein spreading in M83 mice brain revealed by detection of pathological α-synuclein by enhanced ELISA

(2014) Dominique Bétemps et al.   Acta Neuropathologica Communications

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

(2013) Johannes Brettschneider et al.   ANNALS OF NEUROLOGY

β-Amyloid is transmitted via neuronal connections along axonal membranes

(2013) Ha-Lim Song et al.   ANNALS OF NEUROLOGY

Lewy body extracts from Parkinson disease brains trigger α-synuclein pathology and neurodegeneration in mice and monkeys

(2013) Ariadna Recasens et al.   ANNALS OF NEUROLOGY

Molecular Chaperone Functions in Protein Folding and Proteostasis

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

Prion-like spreading of pathological α-synuclein in brain

(2013) Masami Masuda-Suzukake et al.   BRAIN

Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

(2013) J. Ross Buchan et al.   CELL

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

(2013) Ilmin Kwon et al.   CELL

Distinct α-Synuclein Strains Differentially Promote Tau Inclusions in Neurons

(2013) Jing L. Guo et al.   CELL

Altered Ribostasis: RNA-Protein Granules in Degenerative Disorders

(2013) Mani Ramaswami et al.   CELL

The Bacterial Cytoplasm Has Glass-like Properties and Is Fluidized by Metabolic Activity

(2013) Bradley R. Parry et al.   CELL

Caudo-rostral brain spreading of α-synuclein through vagal connections

(2013) Ayse Ulusoy et al.   EMBO Molecular Medicine

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

(2013) Götz Heilbronner et al.   EMBO REPORTS

Spreading of tau pathology in Alzheimer's disease by cell-to-cell transmission

(2013) Nguyen-Vi Mohamed et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

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

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

Stress granules as crucibles of ALS pathogenesis

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

Immune-mediated Mechanisms in the Pathoprogression of Amyotrophic Lateral Sclerosis

(2013) Weihua Zhao et al.   Journal of Neuroimmune Pharmacology

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

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

(2013) Hong Joo Kim et al.   NATURE

Amyotrophic lateral sclerosis—a model of corticofugal axonal spread

(2013) Heiko Braak et al.   Nature Reviews Neurology

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

(2013) Kiran Yanamandra et al.   NEURON

α-Synucleinopathy phenotypes

(2013) Heather McCann et al.   PARKINSONISM & RELATED DISORDERS

Brain homogenates from human tauopathies induce tau inclusions in mouse brain

(2013) Florence Clavaguera et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Environmental exposure to pesticides and cancer risk in multiple human organ systems

(2013) Tesifón Parrón et al.   TOXICOLOGY LETTERS

Structural and functional characterization of two alpha-synuclein strains

(2013) Luc Bousset et al.   Nature Communications

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

(2013) Takashi Nonaka et al.   Cell Reports

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

Parkinson's Disease and Parkinsonism: Neuropathology

(2013) D. W. Dickson   Cold Spring Harbor Perspectives in Medicine

Amyotrophic lateral sclerosis and organ donation: Is there risk of disease transmission?

(2012) Brandon B. Holmes et al.   ANNALS OF NEUROLOGY

Neuron-to-neuron transmission of α-synuclein fibrils through axonal transport

(2012) Eric C. Freundt et al.   ANNALS OF NEUROLOGY

Genetics of Longevity in Model Organisms: Debates and Paradigm Shifts

(2012) David Gems et al.   Annual Review of Physiology

Molecular analysis and biochemical classification of TDP-43 proteinopathy

(2012) Hiroshi Tsuji et al.   BRAIN

Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation

(2012) Mauricio Budini et al.   BRAIN RESEARCH

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease

(2012) Oliver D. King et al.   BRAIN RESEARCH

Mixed Lineage Kinase Domain-like Protein Mediates Necrosis Signaling Downstream of RIP3 Kinase

(2012) Liming Sun et al.   CELL

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

Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS

(2012) Dorothee Dormann et al.   EMBO JOURNAL

Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis

(2012) G. M. Gargiulo-Monachelli et al.   EUROPEAN JOURNAL OF NEUROLOGY

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

(2012) Julien Couthouis et al.   HUMAN MOLECULAR GENETICS

The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth

(2012) Claudia Fallini et al.   HUMAN MOLECULAR GENETICS

Tau Protein Diffuses along the Microtubule Lattice

(2012) Maike H. Hinrichs 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

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

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

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice

(2012) Kelvin C. Luk 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

Regulated protein aggregation: stress granules and neurodegeneration

(2012) Benjamin Wolozin   Molecular Neurodegeneration

Prion-like behaviour and tau-dependent cytotoxicity of pyroglutamylated amyloid-β

(2012) Justin M. Nussbaum et al.   NATURE

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

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

Environmental toxins trigger PD-like progression via increased alpha-synuclein release from enteric neurons in mice

(2012) Francisco Pan-Montojo et al.   Scientific Reports

Risk Factors and Preventive Interventions for Alzheimer Disease

(2011) Martha L. Daviglus   ARCHIVES OF NEUROLOGY

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou 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

TDP-43 Is a Transcriptional Repressor

(2011) Avin S. Lalmansingh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

α-Synuclein propagates from mouse brain to grafted dopaminergic neurons and seeds aggregation in cultured human cells

(2011) Christian Hansen et al.   JOURNAL OF CLINICAL INVESTIGATION

UG Repeats/TDP-43 Interactions near 5′ Splice Sites Exert Unpredictable Effects on Splicing Modulation

(2011) Monica Passoni et al.   JOURNAL OF MOLECULAR BIOLOGY

Prion protein polymerisation triggered by manganese-generated prion protein seeds

(2011) Shirley Hesketh et al.   JOURNAL OF NEUROCHEMISTRY

Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis

(2011) C. Fujimura-Kiyono et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study

(2011) A. Chio et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis

(2011) Sonja Körner et al.   MUSCLE & NERVE

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

(2011) Peter M. Andersen et al.   Nature Reviews Neurology

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

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

Prion-like acceleration of a synucleinopathy in a transgenic mouse model

(2011) Anne-Laure Mougenot et al.   NEUROBIOLOGY OF AGING

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

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

An overview of human prion diseases

(2011) Muhammad Imran et al.   Virology Journal

An overview of animal prion diseases

(2011) Muhammad Imran et al.   Virology Journal

Anti-PrP antibodies detected at terminal stage of prion-affected mouse

(2010) Yukiko Sassa et al.   CELLULAR IMMUNOLOGY

Nuclear factor TDP-43 can affect selected microRNA levels

(2010) Emanuele Buratti et al.   FEBS Journal

Mammalian Prions Generated from Bacterially Expressed Prion Protein in the Absence of Any Mammalian Cofactors

(2010) Jae-Il Kim 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

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Molecular mechanisms of necroptosis: an ordered cellular explosion

(2010) Peter Vandenabeele et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

TAR DNA-binding protein 43 in neurodegenerative disease

(2010) Alice S. Chen-Plotkin et al.   Nature Reviews Neurology

Superoxide Dismutase 1 and tgSOD1G93A Mouse Spinal Cord Seed Fibrils, Suggesting a Propagative Cell Death Mechanism in Amyotrophic Lateral Sclerosis

(2010) Ruth Chia et al.   PLoS One

Identifying the amylome, proteins capable of forming amyloid-like fibrils

(2010) Lukasz Goldschmidt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peripherally Applied A -Containing Inoculates Induce Cerebral  -Amyloidosis

(2010) Y. S. Eisele et al.   SCIENCE

Pattern of spread and prognosis in lower limb-onset ALS

(2009) Martin R. Turner et al.   Amyotrophic Lateral Sclerosis

Lewy body pathology in fetal grafts

(2009) Yaping Chu et al.   Annals of the New York Academy of Sciences

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

(2009) Simon Alberti et al.   CELL

Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis

(2009) G. S. Pesiridis et al.   HUMAN MOLECULAR GENETICS

TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development

(2009) Chantelle F. Sephton 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

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

(2009) Bess Frost et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Seeding induced by α-synuclein oligomers provides evidence for spreading of α-synuclein pathology

(2009) Karin M. Danzer et al.   JOURNAL OF NEUROCHEMISTRY

Incidence of amyotrophic lateral sclerosis in Europe

(2009) G. Logroscino et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Transmission and spreading of tauopathy in transgenic mouse brain

(2009) Florence Clavaguera et al.   NATURE CELL BIOLOGY

Prions hijack tunnelling nanotubes for intercellular spread

(2009) Karine Gousset et al.   NATURE CELL BIOLOGY

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

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

Molecular mechanisms for protein-encoded inheritance

(2009) Jed J W Wiltzius et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

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

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of  -synuclein

(2009) P. Desplats et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyloid seeds formed by cellular uptake, concentration, and aggregation of the amyloid-beta peptide

(2009) X. Hu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Design and construction of diverse mammalian prion strains

(2009) D. W. Colby et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exogenous  -synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells

(2009) K. C. Luk et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Germline P Granules Are Liquid Droplets That Localize by Controlled Dissolution/Condensation

(2009) C. P. Brangwynne et al.   SCIENCE

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

Increased molecular damage and heterogeneity as the basis of aging

(2008) Suresh I.S. Rattan   BIOLOGICAL CHEMISTRY

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation

(2008) Jia-Yi Li et al.   NATURE MEDICINE

Lewy body–like pathology in long-term embryonic nigral transplants in Parkinson's disease

(2008) Jeffrey H Kordower et al.   NATURE MEDICINE

Mitochondrial fragmentation in neurodegeneration

(2008) Andrew B. Knott et al.   NATURE REVIEWS NEUROSCIENCE