Does a loss of TDP-43 function cause neurodegeneration? (Q27026848)

Molecular Neurodegeneration

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

7 April 2017

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Amyotrophic lateral sclerosis

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TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes

7 April 2017

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Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span

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Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

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Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans

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Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice

28 September 2017

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Does α-amino-β-methylaminopropionic acid (BMAA) play a role in neurodegeneration?

28 September 2017

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Making connections: pathology and genetics link amyotrophic lateral sclerosis with frontotemporal lobe dementia.

28 September 2017

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The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span.

28 September 2017

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Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons

28 September 2017

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

28 September 2017

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

28 September 2017

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

28 September 2017

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TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling

28 September 2017

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The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism

28 September 2017

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Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

28 September 2017

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

28 September 2017

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Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy

28 September 2017

1 reference

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Frontotemporal dementia: a review for primary care physicians.

28 September 2017

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Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.

28 September 2017

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TDP-43 proteinopathy and motor neuron disease in chronic traumatic encephalopathy.

28 September 2017

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Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.

28 September 2017

1 reference

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

28 September 2017

1 reference

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The Association of Exposure to Lead, Mercury, and Selenium and the Development of Amyotrophic Lateral Sclerosis and the Epigenetic Implications

28 September 2017

1 reference

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Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism

28 September 2017

1 reference

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The multiple roles of TDP-43 in pre-mRNA processing and gene expression regulation

28 September 2017

1 reference

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

28 September 2017

1 reference

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Neurotoxic effects of TDP-43 overexpression in C. elegans

28 September 2017

1 reference

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TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

28 September 2017

1 reference

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

28 September 2017

1 reference

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Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.

28 September 2017

1 reference

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Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis

28 September 2017

1 reference

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A Drosophila model for TDP-43 proteinopathy

28 September 2017

1 reference

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TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

28 September 2017

1 reference

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Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43 is a developmentally regulated protein essential for early embryonic development

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

DCTN1 mutations in Perry syndrome

28 September 2017

1 reference

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Genetics, environmental factors and the emerging role of epigenetics in neurodegenerative diseases

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching

31 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

31 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates.

31 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Progressive motor weakness in transgenic mice expressing human TDP-43.

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Abnormal TDP-43 expression is identified in the neocortex in cases of dementia pugilistica, but is mainly confined to the limbic system when identified in high and moderate stages of Alzheimer's disease

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury.

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Accumulation of TDP-43 and alpha-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

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

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3419078

Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease

28 November 2018

1 reference

12 December 2020

Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia

1 reference

12 December 2020

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

12 December 2020

Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation

1 reference

12 December 2020

Identifiers

DOI

10.1186/1750-1326-7-27

1 reference

Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

5038982

Dimensions Publication ID

1033773555

0 references

OpenCitations bibliographic resource ID

5038982

1 reference

Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

1 reference

PubMed publication ID

22697423

1 reference