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

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

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

(2014) Staffan Holmqvist et al.   ACTA NEUROPATHOLOGICA

Experimental transmissibility of mutant SOD1 motor neuron disease

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

TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord

(2014) Johannes Brettschneider et al.   ACTA NEUROPATHOLOGICA

Diffusion tensor imaging analysis of sequential spreading of disease in amyotrophic lateral sclerosis confirms patterns of TDP-43 pathology

(2014) J. Kassubek et al.   BRAIN

Focality, stochasticity and neuroanatomic propagation in ALS pathogenesis

(2014) John Ravits   EXPERIMENTAL NEUROLOGY

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

(2014) Jing L Guo et al.   NATURE MEDICINE

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

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

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

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

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

Prion protein oligomer and its neurotoxicity

(2013) P. Huang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid

(2013) Eileen H. Bigio et al.   ACTA NEUROPATHOLOGICA

β-Amyloid is transmitted via neuronal connections along axonal membranes

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

Prion-like spreading of pathological α-synuclein in brain

(2013) Masami Masuda-Suzukake et al.   BRAIN

Molecular Structure of β-Amyloid Fibrils in Alzheimer’s Disease Brain Tissue

(2013) Jun-Xia Lu et al.   CELL

Distinct α-Synuclein Strains Differentially Promote Tau Inclusions in Neurons

(2013) Jing L. Guo et al.   CELL

Delineating the membrane-disrupting and seeding properties of the TDP-43 amyloidogenic core

(2013) Gerard Chun-Hao Liu et al.   CHEMICAL COMMUNICATIONS

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

Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis

(2013) Yoshiaki Furukawa et al.   FEBS LETTERS

TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo

(2013) Darshana K. Choksi 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

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

(2013) J. Janssens et al.   HUMAN MOLECULAR GENETICS

Structural Transformation of the Amyloidogenic Core Region of TDP-43 Protein Initiates Its Aggregation and Cytoplasmic Inclusion

(2013) Lei-Lei Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Can lesions to the motor cortex induce amyotrophic lateral sclerosis?

(2013) Angela Rosenbohm et al.   JOURNAL OF NEUROLOGY

Spreading of amyotrophic lateral sclerosis lesions--multifocal hits and local propagation?

(2013) T. Sekiguchi et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

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

Conformational templating of α-synuclein aggregates in neuronal-glial cultures

(2013) Amanda N Sacino et al.   Molecular Neurodegeneration

Amyotrophic lateral sclerosis—a model of corticofugal axonal spread

(2013) Heiko Braak et al.   Nature Reviews Neurology

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

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

Deciphering amyotrophic lateral sclerosis: What phenotype, neuropathology and genetics are telling us about pathogenesis

(2013) John Ravits et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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

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

Tau oligomers and tau toxicity in neurodegenerative disease

(2012) Sarah M. Ward et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Molecular analysis and biochemical classification of TDP-43 proteinopathy

(2012) Hiroshi Tsuji et al.   BRAIN

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

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

Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region

(2012) Mauricio Budini 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

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

Targeting of Monomer/Misfolded SOD1 as a Therapeutic Strategy for Amyotrophic Lateral Sclerosis

(2012) H.-N. Liu et al.   JOURNAL OF NEUROSCIENCE

The toxic Aβ oligomer and Alzheimer's disease: an emperor in need of clothes

(2012) Iryna Benilova et al.   NATURE NEUROSCIENCE

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

Conserved Acidic Amino Acid Residues in a Second RNA Recognition Motif Regulate Assembly and Function of TDP-43

(2012) Akemi Shodai et al.   PLoS One

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

(2012) K. C. Luk et al.   SCIENCE

The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies

(2012) I.-Fan Wang et al.   Nature Communications

Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy

(2011) Felix Geser et al.   ACTA NEUROPATHOLOGICA

A harmonized classification system for FTLD-TDP pathology

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

The Topography of Brain Microstructural Damage in Amyotrophic Lateral Sclerosis Assessed Using Diffusion Tensor MR Imaging

(2011) E. Canu et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Pathogenic protein seeding in alzheimer disease and other neurodegenerative disorders

(2011) Mathias Jucker et al.   ANNALS OF NEUROLOGY

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

TDP-43: new aspects of autoregulation mechanisms in RNA binding proteins and their connection with human disease

(2011) Emanuele Buratti et al.   FEBS Journal

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

(2011) Jing L. Guo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Frontotemporal Syndromes of ALS. Clinicopathological Correlates

(2011) Michael Joseph Strong et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

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

TDP-43 Autoregulation: Implications for Disease

(2011) Mauricio Budini et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

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

TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6

(2011) Fabienne C Fiesel et al.   Molecular Neurodegeneration

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

The C-Terminal TDP-43 Fragments Have a High Aggregation Propensity and Harm Neurons by a Dominant-Negative Mechanism

(2011) Chunxing Yang et al.   PLoS One

Impaired Structural Motor Connectome in Amyotrophic Lateral Sclerosis

(2011) Esther Verstraete et al.   PLoS One

Implications of the prion-related Q/N domains in TDP-43 and FUS

(2011) Maria Udan et al.   Prion

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

Rapid cell-surface prion protein conversion revealed using a novel cell system

(2011) R. Goold et al.   Nature Communications

Assessment of White Matter Tract Damage in Patients with Amyotrophic Lateral Sclerosis: A Diffusion Tensor MR Imaging Tractography Study: Fig 1.

(2010) F. Agosta et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

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

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

Three- and Four-repeat Tau Coassemble into Heterogeneous Filaments

(2010) Ayisha Siddiqua et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases

(2010) C. Donaghy et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

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

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

Peripherally Applied A -Containing Inoculates Induce Cerebral  -Amyloidosis

(2010) Y. S. Eisele et al.   SCIENCE

The propagation of prion-like protein inclusions in neurodegenerative diseases

(2010) Michel Goedert et al.   TRENDS IN NEUROSCIENCES

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

(2009) Simon Alberti et al.   CELL

Prion protein misfolding and disease

(2009) Roger A Moore et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Expression of TDP-43 C-terminal Fragmentsin VitroRecapitulates Pathological Features of TDP-43 Proteinopathies

(2009) Lionel M. Igaz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons

(2009) Iñigo Casafont et al.   JOURNAL OF STRUCTURAL BIOLOGY

Beyond the prion principle

(2009) Adriano Aguzzi   NATURE

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

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

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

Neurodegenerative Diseases Target Large-Scale Human Brain Networks

(2009) William W. Seeley et al.   NEURON

Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons

(2009) T. Sato et al.   NEUROSCIENCE

Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo

(2009) A. D'Ambrogio et al.   NUCLEIC ACIDS RESEARCH

Amyotrophic lateral sclerosis

(2009) Lokesh C Wijesekera et al.   Orphanet Journal of Rare Diseases

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

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

Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations

(2008) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY

Conformational Diversity of Wild-type Tau Fibrils Specified by Templated Conformation Change

(2008) Bess Frost et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

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

Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review

(2008) M.M. van der Graaff et al.   NEUROMUSCULAR DISORDERS

TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression

(2008) Y. M. Ayala 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

Prion Switching in Response to Environmental Stress

(2008) Jens Tyedmers et al.   PLOS BIOLOGY

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

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