The molecular links between TDP-43 dysfunction and neurodegeneration (Q37593727)

21 August 2017

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title

The molecular links between TDP-43 dysfunction and neurodegeneration (English)

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21 August 2017

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1

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21 August 2017

Francisco E Baralle

2

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21 August 2017

1 January 2009

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21 August 2017

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21 August 2017

66

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21 August 2017

1-34

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A novel CpG-free vertebrate insulator silences the testis-specific SP-10 gene in somatic tissues: role for TDP-43 in insulator function

21 August 2017

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

cis-requirement for the maintenance of round spermatid-specific transcription

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Progranulin in frontotemporal lobar degeneration and neuroinflammation

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

An exonic splicing enhancer offsets the atypical GU-rich 3' splice site of human apolipoprotein A-II exon 3.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Human, Drosophila, and C.elegans TDP43: nucleic acid binding properties and splicing regulatory function

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Structural determinants of the cellular localization and shuttling of TDP-43.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TAR DNA-binding protein-43 in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Alzheimer disease

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicing

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

The many ways to frontotemporal degeneration and beyond

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail: an important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 in neurodegenerative disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

High frequency of TARDBP gene mutations in Italian patients with amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TARDBP (TDP-43) sequence analysis in patients with familial and sporadic ALS: identification of two novel mutations.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 immunoreactivity in neurodegenerative disorders: disease versus mechanism specificity

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 in differential diagnosis of motor neuron disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Messenger-RNA-binding proteins and the messages they carry

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Motor neuron disease and frontotemporal lobar degeneration: a tale of two disorders linked to TDP-43.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Characterization of an RNA granule from developing brain.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

DCTN1 mutations in Perry syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43: a novel neurodegenerative proteinopathy

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer's disease and frontotemporal lobar degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

DEAD-box RNA helicase subunits of the Drosha complex are required for processing of rRNA and a subset of microRNAs

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Clinical and pathological continuum of multisystem TDP-43 proteinopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 A315T mutation in familial motor neuron disease

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

RNA-binding proteins and post-transcriptional gene regulation

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

The Microprocessor complex mediates the genesis of microRNAs

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 is not a common cause of sporadic amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

No TARDBP mutations in a French Canadian population of patients with Parkinson disease

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

hnRNP complexes: composition, structure, and function

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https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Structural insights into TDP-43 in nucleic-acid binding and domain interactions

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 proteinopathies: neurodegenerative protein misfolding diseases without amyloidosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 immunoreactivity in anoxic, ischemic and neoplastic lesions of the central nervous system.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

A protein interaction framework for human mRNA degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 M311V mutation in familial amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

ALS and FTLD: two faces of TDP-43 proteinopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

The molecular genetics and neuropathology of frontotemporal lobar degeneration: recent developments

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

The role of transactive response DNA-binding protein-43 in amyotrophic lateral sclerosis and frontotemporal dementia

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

hnRNP proteins and splicing control.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: protein misfolding diseases without amyloidosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Progranulin and frontotemporal lobar degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 gene analysis in frontotemporal lobar degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 in amyotrophic lateral sclerosis: pathophysiology or patho-babel?

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Loss of presenilin function causes impairments of memory and synaptic plasticity followed by age-dependent neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

No association of TDP-43 with sporadic frontotemporal dementia

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Think locally: control of ubiquitin-dependent protein degradation in neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

A human protein-protein interaction network: a resource for annotating the proteome

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Genetic variability in the regulatory region of presenilin 1 associated with risk for Alzheimer's disease and variable expression

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Pathology and genetics of frontotemporal lobar degeneration: an update.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Higher order arrangement of the eukaryotic nuclear bodies

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43: an emerging new player in neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Pallidonigral TDP-43 pathology in Perry syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

TDP-43 mutation in familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43.

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Epitope mapping of 2E2-D3, a monoclonal antibody directed against human TDP-43

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs

1 reference

https://api.crossref.org/works/10.1016%2FS0065-2660%2809%2966001-6

7 January 2021

10.1016/S0065-2660(09)66001-6

Identifiers

DOI

1 reference

21 August 2017

1 reference

PubMed ID

19737636