From nucleation to widespread propagation: A prion-like concept for ALS. (Q38345753)

2 September 2017

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title

From nucleation to widespread propagation: A prion-like concept for ALS. (English)

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amyotrophic lateral sclerosis

2 September 2017

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Zuzanna Maniecka

1

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PubMed ID

25656065

2 September 2017

Magdalini Polymenidou

series ordinal

2

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2 September 2017

2 February 2015

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2 September 2017

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2 September 2017

207

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2 September 2017

94-105

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Beyond the prion principle

2 September 2017

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Insights into prion strains and neurotoxicity

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mammalian prion biology: one century of evolving concepts

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

The transcellular spread of cytosolic amyloids, prions, and prionoids

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Total RNA sequencing reveals nascent transcription and widespread co-transcriptional splicing in the human brain

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Structural Consequences of Cysteinylation of Cu/Zn-Superoxide Dismutase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Experimental transmissibility of mutant SOD1 motor neuron disease

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: implication for protein aggregation and immune response.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

ALS: a disease of motor neurons and their nonneuronal neighbors

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Onset and progression in inherited ALS determined by motor neurons and microglia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Microglial activation correlates with disease progression and upper motor neuron clinical symptoms in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Eukaryotic stress granules: the ins and outs of translation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mice devoid of PrP are resistant to scrapie

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Transmission and spreading of tauopathy in transgenic mouse brain

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like disorders: blurring the divide between transmissibility and infectivity

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Clinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

RNA molecules stimulate prion protein conversion

7 January 2021

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https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Clinical and pathological continuum of multisystem TDP-43 proteinopathies

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 A315T mutation in familial motor neuron disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

The propagation of prion-like protein inclusions in neurodegenerative diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like activity of Cu/Zn superoxide dismutase: implications for amyotrophic lateral sclerosis.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Lewy bodies in Alzheimer's disease: a neuropathological review of 145 cases using alpha-synuclein immunohistochemistry.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Evaluation of potential infectivity of Alzheimer and Parkinson disease proteins in recipients of cadaver-derived human growth hormone

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Oligodendroglia metabolically support axons and contribute to neurodegeneration.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Trans-synaptic spread of tau pathology in vivo

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

The RRM domain of human fused in sarcoma protein reveals a non-canonical nucleic acid binding site.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like spreading of pathological α-synuclein in brain

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Molecular neuropathology of TDP-43 proteinopathies

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like properties of pathological TDP-43 aggregates from diseased brains

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Seeded aggregation and toxicity of {alpha}-synuclein and tau: cellular models of neurodegenerative diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

A "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transport

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

The seeds of neurodegeneration: prion-like spreading in ALS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like spread of protein aggregates in neurodegeneration

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Misregulated RNA processing in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Novel proteinaceous infectious particles cause scrapie

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Sporadic ALS has compartment-specific aberrant exon splicing and altered cell-matrix adhesion biology

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Motor neuron disease in 2012: Novel causal genes and disease modifiers

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

RNA-mediated neuromuscular disorders

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Focality, stochasticity and neuroanatomic propagation in ALS pathogenesis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Focality of upper and lower motor neuron degeneration at the clinical onset of ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Necroptosis drives motor neuron death in models of both sporadic and familial ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

State of play in amyotrophic lateral sclerosis genetics

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Premorbid weight, body mass, and varsity athletics in ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

An MND/ALS phenotype associated with C9orf72 repeat expansion: abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

C9ORF72 repeat expansions in cases with previously identified pathogenic mutations

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Physical activity and the association with sporadic ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Exogenous α-synuclein fibrils induce Lewy body pathology leading to synaptic dysfunction and neuron death

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Astrocytic TDP-43 pathology in Alexander disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Generating a prion with bacterially expressed recombinant prion protein

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Prospective study of military service and mortality from ALS.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Regulated protein aggregation: stress granules and neurodegeneration

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Hypermethylation of the CpG island near the G4C2 repeat in ALS with a C9orf72 expansion

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

TDP-43 mutation in familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

Non-ATG-initiated translation directed by microsatellite expansions

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.VIRUSRES.2014.12.032

10.1016/J.VIRUSRES.2014.12.032

7 January 2021

Identifiers

DOI

1 reference

2 September 2017

1 reference

PubMed ID

25656065