Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region

scientific article published on 10 January 2012

Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1074/JBC.M111.288720
P932PMC publication ID3293573
P698PubMed publication ID22235134

P50authorCristiana StuaniQ63133919
Emanuele BurattiQ37374238
P2093author name stringFrancisco E Baralle
Corrado Guarnaccia
Valentina Romano
Mauricio Budini
Laura De Conti
P2860cites workNuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skippingQ24291081
Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifsQ24317451
TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expressionQ24318430
Stress granule assembly is mediated by prion-like aggregation of TIA-1Q24559953
Structural insights into TDP-43 in nucleic-acid binding and domain interactionsQ24647225
TDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicingQ24651520
Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene.Q24811044
Delineation of the core aggregation sequences of TDP-43 C-terminal fragmentQ47990018
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degenerationQ48213035
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutaseQ57652550
hnRNP A1 selectively interacts through its Gly-rich domain with different RNA-binding proteinsQ71176264
TDP-43 and FUS: a nuclear affairQ84433614
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosisQ28131672
Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9Q28208568
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 splicingQ28271899
Regulating gene expression through RNA nuclear retentionQ28278213
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementiaQ28294001
Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain TissueQ28475724
Messenger-RNA-binding proteins and the messages they carryQ29616012
hnRNP complexes: composition, structure, and functionQ29619428
A novel CpG-free vertebrate insulator silences the testis-specific SP-10 gene in somatic tissues: role for TDP-43 in insulator functionQ30444718
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6Q30492839
Antagonistic factors control the unproductive splicing of SC35 terminal intron.Q30493457
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesisQ30494776
Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathyQ30499509
Sporadic ALS has compartment-specific aberrant exon splicing and altered cell-matrix adhesion biologyQ33552181
TDP-43 regulates its mRNA levels through a negative feedback loopQ33763346
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanismQ33786698
TAR DNA-binding protein 43 in neurodegenerative diseaseQ33941040
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.Q34068067
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.Q34074385
Nuclear factor TDP-43 can affect selected microRNA levelsQ34112413
Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragmentsQ34137537
Review: transactive response DNA-binding protein 43 (TDP-43): mechanisms of neurodegenerationQ34642738
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.Q34982074
A "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transportQ34998181
TDP-43 is recruited to stress granules in conditions of oxidative insult.Q35003958
The roles of heterogeneous nuclear ribonucleoproteins in tumour development and progressionQ36353980
A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicityQ36609145
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formationQ36741538
Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease.Q36991962
hnRNP proteins and splicing control.Q37124820
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 ProteinopathiesQ37136781
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicityQ37164410
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.Q37171556
TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystanderQ37204062
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathiesQ37220481
Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivoQ37259106
Ribonucleoprotein complexes in neurologic diseasesQ37302017
TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicityQ37339064
Nuclear functions of heterogeneous nuclear ribonucleoproteins A/B.Q37355839
Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosisQ37379471
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseasesQ37409270
The molecular links between TDP-43 dysfunction and neurodegenerationQ37593727
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS).Q37617743
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathyQ37681653
Aberrant RNA processing events in neurological disordersQ37708346
RNA processing pathways in amyotrophic lateral sclerosisQ37720436
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegenerationQ37733168
Protein aggregation and defective RNA metabolism as mechanisms for motor neuron damageQ37734506
TDP-43: a DNA and RNA binding protein with roles in neurodegenerative diseases.Q37769924
The role of RNA processing in the pathogenesis of motor neuron degeneration.Q37774045
Pathogenic TARDBP mutations in amyotrophic lateral sclerosis and frontotemporal dementia: disease-associated pathways.Q37810561
Implications of the prion-related Q/N domains in TDP-43 and FUS.Q37816817
Regulation of gene expression by TDP-43 and FUS/TLS in frontotemporal lobar degenerationQ37827518
RNA-binding proteins and RNA metabolism: a new scenario in the pathogenesis of Amyotrophic lateral sclerosisQ37853914
TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degenerationQ37903412
TDP-43 and FUS/TLS: cellular functions and implications for neurodegeneration.Q37903416
TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathiesQ37904288
RNA-binding proteins with prion-like domains in ALS and FTLD-U.Q37918720
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.Q39584699
Tumor metabolism: hnRNP proteins get in on the actQ39699927
Zinc induces depletion and aggregation of endogenous TDP-43.Q39742732
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.Q39761567
Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.Q39832220
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulinQ39838808
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.Q39839659
Prion-like disorders: blurring the divide between transmissibility and infectivityQ39863503
Structural determinants of the cellular localization and shuttling of TDP-43.Q39924105
Induction of amyloid fibrils by the C-terminal fragments of TDP-43 in amyotrophic lateral sclerosisQ43200632
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).Q46226347
Human, Drosophila, and C.elegans TDP43: nucleic acid binding properties and splicing regulatory functionQ46434717
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behaviorQ47071494
P433issue10
P407language of work or nameEnglishQ1860
P1104number of pages14
P304page(s)7512-7525
P577publication date2012-01-10
P1433published inJournal of Biological ChemistryQ867727
P1476titleCellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region
P478volume287

Reverse relations

cites work (P2860)
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