(Q39240888)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:23172908%20AND%20SRC:MED&resulttype=core&format=json

18 February 2020

title

Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models (English)

1 reference

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18 February 2020

0 references

1

1 reference

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18 February 2020

Alex J Parker

5

1 reference

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18 February 2020

author name string

Babykumari P Chitramuthu

2

1 reference

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18 February 2020

Andrew Bateman

3

1 reference

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18 February 2020

Hugh P J Bennett

4

1 reference

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18 February 2020

publication date

19 November 2012

1 reference

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18 February 2020

published in

Human Molecular Genetics

1 reference

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18 February 2020

volume

22

1 reference

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18 February 2020

issue

4

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18 February 2020

page(s)

782-794

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Protein homeostasis and aging in neurodegeneration

18 February 2020

cites work

1 reference

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Nuclear factor TDP-43 binds to the polymorphic TG repeats in CFTR intron 8 and causes skipping of exon 9: a functional link with disease penetrance

21 January 2018

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The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS).

21 January 2018

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21 January 2018

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Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

21 January 2018

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

21 January 2018

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

21 January 2018

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Does a loss of TDP-43 function cause neurodegeneration?

21 January 2018

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How do the RNA-binding proteins TDP-43 and FUS relate to amyotrophic lateral sclerosis and frontotemporal degeneration, and to each other?

21 January 2018

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Identification of genes that modify ataxin-1-induced neurodegeneration.

21 January 2018

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

21 January 2018

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

21 January 2018

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Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease

21 January 2018

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The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases

21 January 2018

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Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons.

21 January 2018

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Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death

21 January 2018

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Integration of β-catenin, sirtuin, and FOXO signaling protects from mutant huntingtin toxicity

21 January 2018

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Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy

21 January 2018

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TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans

21 January 2018

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

21 January 2018

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Parkinson's disease-associated DJ-1 modulates innate immunity signaling in Caenorhabditis elegans.

21 January 2018

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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.

21 January 2018

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HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration

21 January 2018

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HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin

21 January 2018

1 reference

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Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes

21 January 2018

1 reference

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Structure, function, and mechanism of progranulin; the brain and beyond

21 January 2018

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A neurodegenerative disease mutation that accelerates the clearance of apoptotic cells

21 January 2018

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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

21 January 2018

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Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration

21 January 2018

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

21 January 2018

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Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

21 January 2018

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Polyglutamine protein aggregation and toxicity are linked to the cellular stress response

21 January 2018

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

21 January 2018

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A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

21 January 2018

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FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis

21 January 2018

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Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.

21 January 2018

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Neurotoxic effects of TDP-43 overexpression in C. elegans

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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A Drosophila model for TDP-43 proteinopathy

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Progranulin is expressed within motor neurons and promotes neuronal cell survival

21 January 2018

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Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures

21 January 2018

1 reference

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Progranulin modulates zebrafish motoneuron development in vivoand rescues truncation defects associated with knockdown of Survival motor neuron 1

21 January 2018

1 reference

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Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy

21 January 2018

1 reference

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

21 January 2018

1 reference

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Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin

21 January 2018

1 reference

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

21 January 2018

1 reference

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