The seeds of neurodegeneration: prion-like spreading in ALS.

PubWeight™: 2.03‹?› | Rank: Top 2%

🔗 View Article (PMC 3220614)

Published in Cell on October 28, 2011

Authors

Magdalini Polymenidou1, Don W Cleveland

Author Affiliations

1: Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093-0670, USA.

Articles citing this

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice. J Exp Med (2012) 3.98

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases. Nature (2013) 3.55

The changing scene of amyotrophic lateral sclerosis. Nat Rev Neurosci (2013) 3.16

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain Res (2012) 3.00

The amyloid state and its association with protein misfolding diseases. Nat Rev Mol Cell Biol (2014) 2.99

Controversies and priorities in amyotrophic lateral sclerosis. Lancet Neurol (2013) 2.52

Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study. Lancet Neurol (2014) 1.81

Alzheimer brain-derived tau oligomers propagate pathology from endogenous tau. Sci Rep (2012) 1.77

Prion-like spread of protein aggregates in neurodegeneration. J Exp Med (2012) 1.49

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms. Proc Natl Acad Sci U S A (2014) 1.48

Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A (2013) 1.44

Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43). J Biol Chem (2012) 1.43

TDP-43 aggregation in neurodegeneration: are stress granules the key? Brain Res (2012) 1.35

Emerging mechanisms of molecular pathology in ALS. J Clin Invest (2015) 1.32

Protein aggregation in amyotrophic lateral sclerosis. Acta Neuropathol (2013) 1.32

Molecular nexopathies: a new paradigm of neurodegenerative disease. Trends Neurosci (2013) 1.29

Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans. PLoS One (2013) 1.25

Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.21

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders. Hum Mol Genet (2013) 1.21

Early interneuron dysfunction in ALS: insights from a mutant sod1 zebrafish model. Ann Neurol (2012) 1.17

Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.16

Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice. Proc Natl Acad Sci U S A (2014) 1.14

Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). Acta Neuropathol (2014) 1.14

Neuronal expression of Fig4 is both necessary and sufficient to prevent spongiform neurodegeneration. Hum Mol Genet (2012) 1.13

Superoxide dismutases and superoxide reductases. Chem Rev (2014) 1.12

The prion hypothesis in Parkinson's disease: Braak to the future. Acta Neuropathol Commun (2013) 1.11

Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease. Brain Res (2016) 1.08

Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2014) 1.08

Palmitoylation-induced aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosis. J Biol Chem (2012) 1.05

The role of FUS gene variants in neurodegenerative diseases. Nat Rev Neurol (2014) 1.04

Prions and the potential transmissibility of protein misfolding diseases. Annu Rev Microbiol (2013) 1.04

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation. Hum Mol Genet (2013) 1.02

Advances in motor neurone disease. J R Soc Med (2014) 1.01

Amyloid-like Self-Assembly of a Cellular Compartment. Cell (2016) 1.01

Intracerebral injection of preformed synthetic tau fibrils initiates widespread tauopathy and neuronal loss in the brains of tau transgenic mice. Neurobiol Dis (2014) 0.99

TDP-43 is intercellularly transmitted across axon terminals. J Cell Biol (2015) 0.97

Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6). J Biol Chem (2012) 0.97

De novo generation of infectious prions with bacterially expressed recombinant prion protein. FASEB J (2013) 0.97

TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans. PLoS Genet (2012) 0.97

Delayed disease onset and extended survival in the SOD1G93A rat model of amyotrophic lateral sclerosis after suppression of mutant SOD1 in the motor cortex. J Neurosci (2014) 0.95

Asparaginyl endopeptidase cleaves TDP-43 in brain. Proteomics (2012) 0.93

Network Neurodegeneration in Alzheimer's Disease via MRI Based Shape Diffeomorphometry and High-Field Atlasing. Front Bioeng Biotechnol (2015) 0.92

Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis. Biochim Biophys Acta (2014) 0.92

An imaging and systems modeling approach to fibril breakage enables prediction of amyloid behavior. Biophys J (2013) 0.92

Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis. J Biol Chem (2013) 0.92

Heritable yeast prions have a highly organized three-dimensional architecture with interfiber structures. Proc Natl Acad Sci U S A (2012) 0.91

The contrasting effect of macromolecular crowding on amyloid fibril formation. PLoS One (2012) 0.90

Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key? Exp Neurobiol (2014) 0.88

ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43. PLoS Biol (2016) 0.87

Prions and Protein Assemblies that Convey Biological Information in Health and Disease. Neuron (2016) 0.87

Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure. Oncotarget (2015) 0.87

Early gene expression changes in spinal cord from SOD1(G93A) Amyotrophic Lateral Sclerosis animal model. Front Cell Neurosci (2013) 0.85

Molecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision Medicine. Int J Mol Sci (2016) 0.84

Dysregulation of the autophagy-endolysosomal system in amyotrophic lateral sclerosis and related motor neuron diseases. Neurol Res Int (2012) 0.84

Inhibition of TDP-43 aggregation by nucleic acid binding. PLoS One (2013) 0.83

FTD and ALS--translating mouse studies into clinical trials. Nat Rev Neurol (2015) 0.82

Accumulation of misfolded SOD1 in dorsal root ganglion degenerating proprioceptive sensory neurons of transgenic mice with amyotrophic lateral sclerosis. Biomed Res Int (2014) 0.82

Worming forward: amyotrophic lateral sclerosis toxicity mechanisms and genetic interactions in Caenorhabditis elegans. Front Genet (2014) 0.81

RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration. Neurol Res Int (2012) 0.81

RNA pathogenesis via Toll-like receptor-activated inflammation in expanded repeat neurodegenerative diseases. Front Mol Neurosci (2013) 0.81

Context dependence of protein misfolding and structural strains in neurodegenerative diseases. Biopolymers (2013) 0.80

The influence of pathological mutations and proline substitutions in TDP-43 glycine-rich peptides on its amyloid properties and cellular toxicity. PLoS One (2014) 0.80

Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse model. PLoS Genet (2015) 0.80

A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 0.80

Computational modeling of the relationship between amyloid and disease. Biophys Rev (2012) 0.79

Amyotrophic lateral sclerosis and organ donation: is there risk of disease transmission? Ann Neurol (2012) 0.79

Neuroimaging Endpoints in Amyotrophic Lateral Sclerosis. Neurotherapeutics (2016) 0.79

Molecular and genetic inflammation networks in major human diseases. Mol Biosyst (2016) 0.79

Potential mechanisms and implications for the formation of tau oligomeric strains. Crit Rev Biochem Mol Biol (2016) 0.79

Extracellular vesicles in neurodegenerative disease - pathogenesis to biomarkers. Nat Rev Neurol (2016) 0.79

Oxidation of the tryptophan 32 residue of human superoxide dismutase 1 caused by its bicarbonate-dependent peroxidase activity triggers the non-amyloid aggregation of the enzyme. J Biol Chem (2014) 0.79

ZNStress: a high-throughput drug screening protocol for identification of compounds modulating neuronal stress in the transgenic mutant sod1G93R zebrafish model of amyotrophic lateral sclerosis. Mol Neurodegener (2016) 0.78

Human protein aging: modification and crosslinking through dehydroalanine and dehydrobutyrine intermediates. Aging Cell (2013) 0.78

Primary cilia and autophagic dysfunction in Huntington's disease. Cell Death Differ (2015) 0.78

Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot. Acta Neuropathol Commun (2014) 0.78

Histochemical approaches to assess cell-to-cell transmission of misfolded proteins in neurodegenerative diseases. Eur J Histochem (2013) 0.77

Key Points Concerning Amyloid Infectivity and Prion-Like Neuronal Invasion. Front Mol Neurosci (2016) 0.77

What amyloid ligands can tell us about molecular polymorphism and disease. Neurobiol Aging (2016) 0.77

Cupric ions induce the oxidation and trigger the aggregation of human superoxide dismutase 1. PLoS One (2013) 0.77

Structural switching of Cu,Zn-superoxide dismutases at loop VI: insights from the crystal structure of 2-mercaptoethanol-modified enzyme. Biosci Rep (2012) 0.77

ALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium Triad. Front Cell Neurosci (2015) 0.77

Pseudobulbar affect (PBA) in an incident ALS cohort: results from the Apulia registry (SLAP). J Neurol (2015) 0.77

In vitro prion-like behaviour of TDP-43 in ALS. Neurobiol Dis (2016) 0.76

Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting neuronal cell loss, oxidative damage and levels of non-native hSOD1(G93A) forms. PLoS One (2013) 0.76

Variant-specific prion interactions: Complicating factors. Cell Logist (2013) 0.76

Side of limb-onset predicts laterality of gray matter loss in amyotrophic lateral sclerosis. Biomed Res Int (2014) 0.76

Nucleocytoplasmic transport in C9orf72-mediated ALS/FTD. Nucleus (2016) 0.75

Formation and spreading of TDP-43 aggregates in cultured neuronal and glial cells demonstrated by time-lapse imaging. PLoS One (2017) 0.75

Prions on the run: How extracellular vesicles serve as delivery vehicles for self-templating protein aggregates. Prion (2017) 0.75

RNA Granules and Diseases: A Case Study of Stress Granules in ALS and FTLD. Adv Exp Med Biol (2016) 0.75

A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects. Dis Model Mech (2016) 0.75

The role of crowded physiological environments in prion and prion-like protein aggregation. Int J Mol Sci (2013) 0.75

Bee venom effects on ubiquitin proteasome system in hSOD1(G85R)-expressing NSC34 motor neuron cells. BMC Complement Altern Med (2013) 0.75

Prognostic role of "prion-like propagation" in SOD1-linked familial ALS: an alternative view. Front Cell Neurosci (2014) 0.75

Acute Traumatic Brain Injury Does Not Exacerbate Amyotrophic Lateral Sclerosis in the SOD1 (G93A) Rat Model(1,2,3). eNeuro (2015) 0.75

Metabolism of minor isoforms of prion proteins: Cytosolic prion protein and transmembrane prion protein. Neural Regen Res (2013) 0.75

Perspective Insights of Exosomes in Neurodegenerative Diseases: A Critical Appraisal. Front Aging Neurosci (2017) 0.75

Structural Rearrangement upon Fragmentation of the Stability Core of the ALS-Linked Protein TDP-43. Biophys J (2017) 0.75

Articles cited by this

(truncated to the top 100)

Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol (1991) 49.03

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96

Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature (1993) 21.65

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science (2008) 14.09

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (2009) 13.45

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (2009) 12.99

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun (2006) 10.69

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet (2008) 9.51

Onset and progression in inherited ALS determined by motor neurons and microglia. Science (2006) 9.42

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci (2007) 7.77

Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol (2009) 7.50

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation. Nat Med (2008) 6.90

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synuclein. Proc Natl Acad Sci U S A (2009) 6.87

In vitro generation of infectious scrapie prions. Cell (2005) 6.86

Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson's disease. Nat Med (2008) 6.86

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science (2006) 6.47

Generating a prion with bacterially expressed recombinant prion protein. Science (2010) 6.26

Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. Nat Neurosci (2007) 6.08

Stress granule assembly is mediated by prion-like aggregation of TIA-1. Mol Biol Cell (2004) 6.04

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01

Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules. J Cell Biol (2000) 5.99

MAVS forms functional prion-like aggregates to activate and propagate antiviral innate immune response. Cell (2011) 5.91

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci (2008) 5.89

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol (2009) 5.65

TDP-43 A315T mutation in familial motor neuron disease. Ann Neurol (2008) 5.58

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis. Lancet Neurol (2008) 5.33

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature (2010) 5.26

Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science (1998) 5.23

RNA granules: post-transcriptional and epigenetic modulators of gene expression. Nat Rev Mol Cell Biol (2009) 4.57

Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature (1966) 4.29

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci (2010) 4.25

A neuronal isoform of the aplysia CPEB has prion-like properties. Cell (2003) 4.16

RNA molecules stimulate prion protein conversion. Nature (2003) 4.06

Peripherally applied Abeta-containing inoculates induce cerebral beta-amyloidosis. Science (2010) 3.98

Prions as adaptive conduits of memory and inheritance. Nat Rev Genet (2005) 3.97

Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation. Cell Stem Cell (2008) 3.97

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci (2005) 3.69

The transcellular spread of cytosolic amyloids, prions, and prionoids. Neuron (2009) 3.68

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. EMBO J (2010) 3.58

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates. Nat Cell Biol (2009) 3.55

α-Synuclein propagates from mouse brain to grafted dopaminergic neurons and seeds aggregation in cultured human cells. J Clin Invest (2011) 3.53

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2008) 3.46

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol (2011) 3.45

Emerging principles of conformation-based prion inheritance. Annu Rev Biochem (2004) 3.36

Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells. Cell Stem Cell (2008) 3.34

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A (2006) 3.30

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem (2009) 3.29

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A (2011) 3.23

Molecular mechanisms of prion pathogenesis. Annu Rev Pathol (2008) 3.05

A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions. Proc Natl Acad Sci U S A (2003) 2.94

TDP-43 mutation in familial amyotrophic lateral sclerosis. Ann Neurol (2008) 2.92

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91

TDP-43 regulates its mRNA levels through a negative feedback loop. EMBO J (2010) 2.90

Seeding of normal Tau by pathological Tau conformers drives pathogenesis of Alzheimer-like tangles. J Biol Chem (2011) 2.86

Idiopathic Parkinson's disease: possible routes by which vulnerable neuronal types may be subject to neuroinvasion by an unknown pathogen. J Neural Transm (Vienna) (2003) 2.75

Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS Biol (2011) 2.70

Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation. Cell (2010) 2.65

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. J Clin Invest (2011) 2.58

TDP-43 is recruited to stress granules in conditions of oxidative insult. J Neurochem (2009) 2.57

Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One (2010) 2.49

Evidence for seeding of beta -amyloid by intracerebral infusion of Alzheimer brain extracts in beta -amyloid precursor protein-transgenic mice. J Neurosci (2000) 2.41

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. Am J Pathol (2008) 2.41

Conformational diversity of wild-type Tau fibrils specified by templated conformation change. J Biol Chem (2008) 2.34

Prion strain discrimination in cell culture: the cell panel assay. Proc Natl Acad Sci U S A (2007) 2.30

Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29

Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation. Proc Natl Acad Sci U S A (2009) 2.27

Prions as protein-based genetic elements. Annu Rev Microbiol (2002) 2.21

Prion-like acceleration of a synucleinopathy in a transgenic mouse model. Neurobiol Aging (2011) 2.18

The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response. BMC Cell Biol (2008) 2.04

FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis. Ann Neurol (2010) 1.97

Seeded aggregation and toxicity of {alpha}-synuclein and tau: cellular models of neurodegenerative diseases. J Biol Chem (2010) 1.92

Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. J Neurosci (2008) 1.91

Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients. PLoS One (2010) 1.89

The benefits and limitations of animal models for translational research in neurodegenerative diseases. Nat Med (2010) 1.86

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A (2011) 1.82

Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease. Hum Mol Genet (2009) 1.79

Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury. Brain Res (2008) 1.77

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1. Hum Mol Genet (2011) 1.75

Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. Ann Neurol (2010) 1.71

Cell biology: Beyond the prion principle. Nature (2009) 1.70

Focality of upper and lower motor neuron degeneration at the clinical onset of ALS. Neurology (2007) 1.67

Cerebral amyloid angiopathy and its relationship to Alzheimer's disease. Acta Neuropathol (2008) 1.60

TDP-43-mediated neuron loss in vivo requires RNA-binding activity. PLoS One (2010) 1.58

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity. Nat Struct Mol Biol (2011) 1.58

Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease. J Neuropathol Exp Neurol (2008) 1.56

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathol (2010) 1.49

A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions. J Biol Chem (2011) 1.48

Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43. J Biol Chem (2010) 1.47

RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells. J Biol Chem (2007) 1.46

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo. Proc Natl Acad Sci U S A (2011) 1.38

Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem. Neurology (2007) 1.38

Induction of beta (A4)-amyloid in primates by injection of Alzheimer's disease brain homogenate. Comparison with transmission of spongiform encephalopathy. Mol Neurobiol (1994) 1.31

A "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transport. J Biol Chem (2011) 1.27

Differential loss of striatal projection systems in Huntington's disease: a quantitative immunohistochemical study. J Chem Neuroanat (2004) 1.27

Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis. PLoS One (2010) 1.25

Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease. Acta Neuropathol (2008) 1.22

Cross-seeding fibrillation of Q/N-rich proteins offers new pathomechanism of polyglutamine diseases. J Neurosci (2009) 1.16

FUS-immunoreactive intranuclear inclusions in neurodegenerative disease. Brain Pathol (2009) 1.15

Articles by these authors

(truncated to the top 100)

Onset and progression in inherited ALS determined by motor neurons and microglia. Science (2006) 9.42

ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron (2006) 7.82

On the road to cancer: aneuploidy and the mitotic checkpoint. Nat Rev Cancer (2005) 7.53

Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci (2004) 6.99

The human CENP-A centromeric nucleosome-associated complex. Nat Cell Biol (2006) 6.85

Boveri revisited: chromosomal instability, aneuploidy and tumorigenesis. Nat Rev Mol Cell Biol (2009) 6.43

Aneuploidy acts both oncogenically and as a tumor suppressor. Cancer Cell (2006) 6.37

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci (2008) 5.89

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol (2009) 5.65

Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71

Propagation of centromeric chromatin requires exit from mitosis. J Cell Biol (2007) 4.60

Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron (2012) 4.48

A standardized kinesin nomenclature. J Cell Biol (2004) 4.43

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A (2002) 4.37

Structural determinants for generating centromeric chromatin. Nature (2004) 4.25

Centromere-specific assembly of CENP-a nucleosomes is mediated by HJURP. Cell (2009) 4.22

Does aneuploidy cause cancer? Curr Opin Cell Biol (2006) 3.89

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci (2008) 3.78

Decoding the links between mitosis, cancer, and chemotherapy: The mitotic checkpoint, adaptation, and cell death. Cancer Cell (2005) 3.69

Lethality to human cancer cells through massive chromosome loss by inhibition of the mitotic checkpoint. Proc Natl Acad Sci U S A (2004) 3.69

Dynamics of centromere and kinetochore proteins; implications for checkpoint signaling and silencing. Curr Biol (2004) 3.65

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63

Unattached kinetochores catalyze production of an anaphase inhibitor that requires a Mad2 template to prime Cdc20 for BubR1 binding. Dev Cell (2009) 3.36

Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron (2002) 3.31

Unstable kinetochore-microtubule capture and chromosomal instability following deletion of CENP-E. Dev Cell (2002) 3.23

Centromere-associated protein-E is essential for the mammalian mitotic checkpoint to prevent aneuploidy due to single chromosome loss. J Cell Biol (2003) 3.15

Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat Neurosci (2002) 3.09

Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease. Nat Neurosci (2007) 3.02

Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest (2006) 3.00

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration. Proc Natl Acad Sci U S A (2013) 2.95

Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 2.94

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci (2012) 2.93

Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell (2012) 2.83

Centromere identity maintained by nucleosomes assembled with histone H3 containing the CENP-A targeting domain. Mol Cell (2007) 2.76

Epigenetic centromere propagation and the nature of CENP-a nucleosomes. Cell (2011) 2.74

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci (2013) 2.71

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Proc Natl Acad Sci U S A (2010) 2.65

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48

Activating and silencing the mitotic checkpoint through CENP-E-dependent activation/inactivation of BubR1. Cell (2003) 2.47

Cep152 interacts with Plk4 and is required for centriole duplication. J Cell Biol (2010) 2.45

ZW10 links mitotic checkpoint signaling to the structural kinetochore. J Cell Biol (2005) 2.36

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A (2008) 2.35

Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2007) 2.25

An epigenetic mark generated by the incorporation of CENP-A into centromeric nucleosomes. Proc Natl Acad Sci U S A (2007) 2.23

An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med (2007) 2.20

Aneuploidy: instigator and inhibitor of tumorigenesis. Cancer Res (2007) 2.12

Microtubule capture by CENP-E silences BubR1-dependent mitotic checkpoint signaling. J Cell Biol (2005) 2.11

Catalytic assembly of the mitotic checkpoint inhibitor BubR1-Cdc20 by a Mad2-induced functional switch in Cdc20. Mol Cell (2013) 2.06

Polo-like kinase 4 kinase activity limits centrosome overduplication by autoregulating its own stability. J Cell Biol (2010) 2.05

Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol (2003) 2.02

Aurora kinases and protein phosphatase 1 mediate chromosome congression through regulation of CENP-E. Cell (2010) 2.00

Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.97

CENP-A-containing nucleosomes: easier disassembly versus exclusive centromeric localization. J Mol Biol (2007) 1.93

Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab (2012) 1.92

Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J Clin Invest (2009) 1.86

Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A (2008) 1.84

Losing balance: the origin and impact of aneuploidy in cancer. EMBO Rep (2012) 1.81

Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A (2007) 1.79

CENP-E combines a slow, processive motor and a flexible coiled coil to produce an essential motile kinetochore tether. J Cell Biol (2008) 1.77

Removal of Spindly from microtubule-attached kinetochores controls spindle checkpoint silencing in human cells. Genes Dev (2010) 1.66

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron (2010) 1.65

A two-step mechanism for epigenetic specification of centromere identity and function. Nat Cell Biol (2013) 1.52

Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci (2013) 1.51

Medicine. Treating neurodegenerative diseases with antibiotics. Science (2005) 1.51

Prion-like spread of protein aggregates in neurodegeneration. J Exp Med (2012) 1.49

Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F. EMBO J (2005) 1.48

NuMA after 30 years: the matrix revisited. Trends Cell Biol (2010) 1.48

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A (2013) 1.48

Double-strand DNA breaks recruit the centromeric histone CENP-A. Proc Natl Acad Sci U S A (2009) 1.47

Requirements for NuMA in maintenance and establishment of mammalian spindle poles. J Cell Biol (2009) 1.47

Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A (2008) 1.45

Human Zwint-1 specifies localization of Zeste White 10 to kinetochores and is essential for mitotic checkpoint signaling. J Biol Chem (2004) 1.44

Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann Neurol (2005) 1.44

Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A (2013) 1.44

Chromosomal instability by inefficient Mps1 auto-activation due to a weakened mitotic checkpoint and lagging chromosomes. PLoS One (2008) 1.43

Misregulated RNA processing in amyotrophic lateral sclerosis. Brain Res (2012) 1.42

NF-M is an essential target for the myelin-directed "outside-in" signaling cascade that mediates radial axonal growth. J Cell Biol (2003) 1.41

The role of aneuploidy in promoting and suppressing tumors. J Cell Biol (2009) 1.39

Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS. Mol Ther (2013) 1.36

Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS One (2008) 1.34

Inducible, reversible system for the rapid and complete degradation of proteins in mammalian cells. Proc Natl Acad Sci U S A (2012) 1.33

Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A (2009) 1.33

Chromoanagenesis and cancer: mechanisms and consequences of localized, complex chromosomal rearrangements. Nat Med (2012) 1.32

Chromosome missegregation rate predicts whether aneuploidy will promote or suppress tumors. Proc Natl Acad Sci U S A (2013) 1.28

Human condensin function is essential for centromeric chromatin assembly and proper sister kinetochore orientation. PLoS One (2009) 1.27

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A (2010) 1.26

A chemical tool box defines mitotic and interphase roles for Mps1 kinase. J Cell Biol (2010) 1.25

Error-prone mammalian female meiosis from silencing the spindle assembly checkpoint without normal interkinetochore tension. Proc Natl Acad Sci U S A (2012) 1.23

The autoregulated instability of Polo-like kinase 4 limits centrosome duplication to once per cell cycle. Genes Dev (2012) 1.23

The aneuploidy paradox in cell growth and tumorigenesis. Cancer Cell (2008) 1.19

Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A (2005) 1.19

Modest loss of peripheral axons, muscle atrophy and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1. J Neurochem (2008) 1.18

C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A (2013) 1.17

Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem (2005) 1.16

Hindlimb gait defects due to motor axon loss and reduced distal muscles in a transgenic mouse model of Charcot-Marie-Tooth type 2A. Hum Mol Genet (2007) 1.16

Cell biology: nondisjunction, aneuploidy and tetraploidy. Nature (2006) 1.15

Slow axonal transport: fast motors in the slow lane. Curr Opin Cell Biol (2002) 1.14

Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice. Proc Natl Acad Sci U S A (2014) 1.14