TDP-43
Hsp90 and its Co-chaperones Modify TDP-43 Localization, Aggregation, and Toxicity
... protein 43 (TDP-43), is a 43 kDa DNA/RNA-binding protein of 414 amino acids encoded by the TAR DNA-binding protein (TARDBP) gene located on chromosome ...[67]. TDP-43 contains a ...
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FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases
... and TDP-43 positive neuronal inclusions; (FTLD- MND): Frontotemporal lobar degeneration associated with motor neuron disease; (ALS): Amyotrophic lateral sclerosis; (TDP-43): Transactive ...
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Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS
... the TDP-43 Q331K trans- genic animals compared to their non-transgenic and TDP-43 WT aged –matched ...in TDP-43 WTxQ331K mice at 8 weeks (Additional file 1: Figure S8), with no ...
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Dioxins and related environmental contaminants increase TDP-43 levels
... of TDP-43 protein levels plays a key role in the pathophysiology of ALS, we investigated whether AHR agonists increased TDP-43 protein as well as ...monomeric TDP-43 ...the ...
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Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation
... encodes TDP- 43, occur in about 3% of sporadic and familial cases of ALS [3] and are mostly found in cases with a usually predominant lower motor neuron involvement and in a few patients with cognitive ...
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Review Article TDP-43 in aging and Alzheimer’s disease – a review
... [65] Van Deerlin VM, Sleiman PM, Martinez-Lage M, Chen-Plotkin A, Wang LS, Graff-Radford NR, Dickson DW, Rademakers R, Boeve BF, Grossman M, Arnold SE, Mann DM, Pickering- Brown SM, Seelaar H, Heutink P, van Swieten JC, ...
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Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
... NTG, TDP-43(A315T) and TDP-43(A315T)xGRN mice (n = 3 per ...in TDP-43(A315T) mice was confirmed and this was not altered by PGRN overexpression (Additional file 1: Fig- ure ...
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Reducing TDP-43 aggregation does not prevent its cytotoxicity
... which TDP-43 is involved in neuronal death and degeneration remains ...of TDP- 43 proteinopathies is the cytoplasmic inclusions of TDP- 43 ...diseases, TDP-43 ...
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Distinct TDP-43 inclusion morphologies in frontotemporal lobar degeneration with and without amyotrophic lateral sclerosis
... circumferential TDP-43 inclusions in the anterior cingulate cortex of FTLD cases, and signifi- cant amounts of circumferential but not rounded TDP- 43 inclusions in the anterior cingulate ...
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Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients
... Amyotrophic lateral sclerosis (ALS) is an adult-onset disease characterized by the selective degeneration of motor neurons in the brain and spinal cord progressively leading to paralysis and death. Current diagnosis of ...
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Dysregulation of the ALS associated gene TDP 43 leads to neuronal death and degeneration in mice
... plasmic TDP-43 inclusions and since previous studies have shown that hTDP-43-WT or hTDP-43-ΔNLS expression reduces endog- enous mTDP-43 levels in cultured nonneuronal cells (11), we ...
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Pathological phosphorylation of tau and TDP-43 by TTBK1 and TTBK2 drives neurodegeneration
... Human TDP-43 was detected with the commercially available monoclonal antibody ab57105 (Abcam, 1:2500) directed at human TDP-43 amino acids ...1-261. TDP-43 phosphorylated at ...
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Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration
... Little is known concerning any specific role for hnRNP E2 within the nervous system in health or neurodegener- ative disease, beyond that of control of mRNA stability and mRNA translation. Broderick and coworkers ...
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Age-dependent changes in TDP-43 levels in a mouse model of Alzheimer disease are linked to Aβ oligomers accumulation
... with TDP-43 immunoreactivity ...mulation, TDP-43 immunoreactivity is not related with Braak neurofibrillary tau pathology ...mice, TDP-43 levels did not correlate with tau ...
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Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
... nuclear TDP-43. The manner through which TDP-43 causes neurodegenera- tion has not been identified; however, recently TDP-43 mouse models generated by our group and others ...
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Therapeutic effect of berberine on TDP-43-related pathogenesis in FTLD and ALS
... truncated TDP-43 fragments through activat- ing the autophagic function in cellular culture model, implying its potential neuroprotective effects on neu- rodegenerative diseases with TDP-43 ...
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Quantitative analysis of cryptic splicing associated with TDP-43 depletion
... of TDP-43 but not FUS results in cryptic exons We compared the involvement of two ALS-linked RNA-binding proteins in cryptic splicing: TDP-43 and ...available TDP-43 depletion ...
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Incidence and extent of TDP-43 accumulation in aging human brain
... of TDP-43 immunoreactive structures in normal ...for TDP-43 in neurologically normal individuals ranging from 23 to 94 years old at death ...showed TDP-43 immu- noreactivity in ...
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TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6
... that TDP-43 knockdown significantly impairs the removal of a toxic, aggregating polyQ ataxin-3 fusion protein in an HDAC6- dependent ...of TDP-43 and its target HDAC6 on neurite ...silenced ...
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Combined use of CSF NfL and CSF TDP 43 improves diagnostic performance in ALS:A comprehensive analysis on diagnostic and prognostic significance of plasma and CSF NfL, TDP 43, and tau
... Combined analysis of validation and discovery cohorts regarding plasma TDP-43,... Kasai and Kojima, et al.[r] ...
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