TDP-43 proteinopathy
Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer’s disease and cerebrovascular disease pathologies
... protein 43 (TDP-43) proteinopathy is a common brain pathology in elderly persons, but much remains to be learned about this high-morbidity ...with TDP-43 pathology in aged ...
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Pathological tau deposition in Motor Neurone Disease and frontotemporal lobar degeneration associated with TDP-43 proteinopathy
... both TDP-43 and tau path- ology in MND patients with and without cognitive impair- ...and TDP-43 pathology is present, and that widespread (astrocytic) tau pathology may be fundamental to ...
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Spatial patterns of phosphorylation-dependent TDP-43-immunoreactive neuronal cytoplasmic inclusions (NCI) in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)
... A major feature of the anatomical structure of the cerebral cortex is the replicated local neural circuit (represented by ‘columns’ or ‘modules’) (Hiorns et al. Table 2. Summary of spatial patterns exhibited by the ...
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Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy
... driving TDP-43 proteinopathy in ...and TDP-43 pathology, and to determine the critical pathologic features that need to be targeted in order to rescue neuronal loss and behavioral ...
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RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy
... mediating TDP-43/RNA interactions whereas RRM2 has a lower affinity for RNA and has a predomin- antly regulatory function (Buratti and Baralle, 2001; Lukavsky et ...of TDP-43 to its target RNA, ...
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Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy
... The precise mechanisms of neuronal injury in C9ORF72- disease appear complex, and are likely to involve RNA gain-of-function toxicity mediated by sense and antisense transcription of the GGGGCC repeat expansion (reviewed ...
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A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)
... Halliday GM, Bird TD, Ince PG, Shaw PJ, Cairns NJ, Morris JC, McLean CA, DeCarli C, Ellis WG, Freeman SH, Frosch MP, Growdon JH, Perl DP, Sano M, Bennett DA, Schneider JA, Beach TG, REiman EM, Woodruff BK, Cummings J, ...
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Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure
... of 43 kDa (TDP-43) aggregates accumulation throughout the nervous ...of TDP-43 positive inclusion in the regional spread of ALS symptoms, whether and how TDP-43 aggregates ...
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Hsp90 and its Co-chaperones Modify TDP-43 Localization, Aggregation, and Toxicity
... i.e. TDP-43 toxicity (Figure 21A), which may be due to the low transfection efficiency in our HEK transfection ...between TDP-43 and Sti1. In wild type HEK cells (Figure 21B), ...
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TDP-43 Potentiates Alpha-synuclein Toxicity to Dopaminergic Neurons in Transgenic Mice
... While TDP-43 proteinopathy is considered a pathologic hallmark of sporadic amyotrophic lateral sclerosis and frontotemporal lobe degeneration, α-synuclein is a major component of Lewy body ...
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Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis
... of TDP-43 proteinopathy are clearance of the protein from the nucleus and its accumulation and aggregation in the cytoplasm [5, ...of TDP-43 function in two stable cell ...50% ...
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Does a loss of TDP-43 function cause neurodegeneration?
... protein 43 kDa (TDP-43) was discovered to be in the intracellular aggregates in the degenerating cells in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), two fatal ...
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Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage
... tein 43 (TDP-43). Generally localized in the nucleus, TDP-43 belongs to the family of heterogeneous nuclear ribonuclear proteins that are highly conserved in differ- ent species ...
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Distinct TDP-43 inclusion morphologies in frontotemporal lobar degeneration with and without amyotrophic lateral sclerosis
... by TDP-43 neuronal inclusions in surviving motor ...pathological TDP-43 inclusions and the FTLD-ALS continuum, FTLD-TDP type C cases were ...
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Reducing TDP-43 aggregation does not prevent its cytotoxicity
... mutated TDP-43 (Δ187-192) can induce TDP-43 aggregation and cell death in HeLa cells ...many TDP-43 mu- tations have been found, the majority of ALS and FTLD-U cases have no ...
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Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo
... Since no defects in mitochondrial respiration and ATP synthesis were detected at presymptomatic (45 days) and at symptomatic (90 days) ages, in order to avoid poten- tially confounding effects due to major changes in ...
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Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP
... [12,23-28]. TDP-43 is localized to cytoplasmic inclusions in post-mortem tissue from ALS patients and since some of these inclusions are also positive for SG markers, some have suggested that deregulation ...
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Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
... A pathway analysis of these genes did not provide evi- dence for a protective effect of PGRN on one specific pathway, it rather hinted at a pleiotropic effect of PGRN on different pathways, some with possible importance ...
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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
... of TDP-43 from neu- rons containing cytoplasmic inclusions, similar to that seen in two-thirds of surviving aggregate containing cells in the TDP-43 WTxQ331K animals, has been reported in ALS ...
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Review Article Neuropathology of non-Alzheimer degenerative disorders
... detected TDP-43 immunoreactivity, most often similar to Mackenzie type 1, in over 70% of cases ...FTLD-TDP, TDP-43 pathology in HpScl may be limited to limbic lobe structures, ...
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