[PDF] Top 20 El control de racionalidad en el gasto público
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TDP-43 Potentiates Alpha-synuclein Toxicity to Dopaminergic Neurons in Transgenic Mice
... normal TDP-43 transgenic mouse colony, we crossed the transgenic founder with C57BL6/J mice newly purchased from Jackson La- ...male transgenic mice of the first three ... See full document
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Conditional transgenic mice expressing C-terminally truncated human α-synuclein (αSyn119) exhibit reduced striatal dopamine without loss of nigrostriatal pathway dopaminergic neurons
... of transgenic mice have been created that express wild-type or mutant human α-synuclein in neurons from an array of different heterologous promoter ...α- synuclein-related neurological ... See full document
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No apparent transmission of transgenic α–synuclein into nigrostriatal dopaminergic neurons in multiple mouse models
... (SNCA) transgenic mice under the control of tetracyc- line operator (tetO): ...of transgenic SNCA assumes to be regulated by the tetra- cycline transactivator (tTA) in a “tet–off” gene expres- sion ... See full document
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Sustained Expression of TDP-43 and FUS in Motor Neurons in Rodent's Lifetime
... vulnerable neurons and an in- crease in gene expression is sufficient to induce neu- ...rodegeneration. Alpha-synuclein is linked to Parkin- son’s disease, an age-related neurodegenerative dis- ease ... See full document
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Mice deficient in the C-terminal domain of TAR DNA-binding protein 43 develop age-dependent motor dysfunction associated with impaired Notch1−Akt signaling pathway
... or TDP-ΔC mice were sonicated in ice-cold lysis buffer (50 mM Tris-HCl, 150 mM NaCl, 1 mM ethylenediaminetetraacetic acid (EDTA), 1%(v/v) Triton X-100) supplemented with protease inhibitor cocktail ... See full document
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Dynamic transport and localization of alpha-synuclein in primary hippocampal neurons
... Each construct was transfected into primary hippo- campal neurons and visualized at 17-21 DIV. ASGFP was observed to localize at discrete punctae along axo- nal processes, with minimal signal detected in interven- ... See full document
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Alpha-Synuclein Toxicity is Caused by Mitochondrial Dysfunction
... aSyn toxicity and aggregation (Figure 9, 10). This increased toxicity and aggregation is not caused merely by higher levels of aSyn protein levels, as western blots have confirmed that our aSyn expression ... See full document
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Hsp90 and its Co-chaperones Modify TDP-43 Localization, Aggregation, and Toxicity
... in neurons of ALS patient brains and spinal ...as TDP-43 proteinopathy, the mislocalization of TDP-43 from the cell nucleus and the formation of aggregates in the ...encoding ... See full document
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Dysregulation of the ALS associated gene TDP 43 leads to neuronal death and degeneration in mice
... protein 43 (TDP-43). TDP-43 is normally localized in the nucleus with roles in the regulation of gene expression, and pathological cytoplasmic aggregates are associated with depletion ... See full document
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RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy
... protein toxicity are not clear, they have a longer half-life than wild-type TDP-43 and are more prone to form cytoplasmic aggregates (Barmada et ...both TDP-43 RRM domains bound to a ... See full document
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Transcriptomopathies of pre- and post-symptomatic frontotemporal dementia-like mice with TDP-43 depletion in forebrain neurons
... with transgenic overexpression of TDP-43, knock-out/ knock-down of Tardbp gene expres- sion often serve as the biological system for exploring the physiological functions of TDP-43 and ... See full document
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Znf179 E3 ligase-mediated TDP-43 polyubiquitination is involved in TDP-43- ubiquitinated inclusions (UBI) (+)-related neurodegenerative pathology
... To quantify the protein expression levels of 26S prote- asome subunits in wild-type and Znf179-knockout mice brain, we used label-free quantitative MS. Hippocampus brain tissue dissected from wild-type and ... See full document
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The electrophysiological impact of oligomeric alpha Synuclein on thick tufted layer 5 pyramidal neurons in the neocortex of mice
... Annular oligomers in particular have been shown to form membrane inserting pore- complexes that can induce cell death through various mechanisms including membrane permeabilisation (Volles and Lansbury, 2002; Schmidt et ... See full document
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Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer’s disease and cerebrovascular disease pathologies
... phosphorylated TDP-43 de- posits in cytoplasmic (where it may appear like speckles, skeins, or tangles), intranuclear, perivascular, and/or neurite-like ...structures. TDP-43 pathology may ... See full document
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Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
... mutant TDP-43(A315T) overexpres- ...overexpression mice to TDP-43(A315T) mice, we could indeed observe a significant improvement in the survival of these mu- tant ... See full document
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Mutant TDP 43 in motor neurons promotes the onset and progression of ALS in rats
... mutant TDP-43 expression results in par- tial recovery of motor function in ...mutant TDP-43 transgene (hTDP-43) as early as 5 days after Dox was ... See full document
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Prion-like propagation of human brain-derived alpha-synuclein in transgenic mice expressing human wild-type alpha-synuclein
... pathological alpha-synuclein in the brains of patients is progressive and can be classified into stages [3, ...of alpha-synuclein in embryonic dopamine neurons grafted into the striatum ... See full document
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Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis
... that TDP-43 knockdown in the adult murine nervous system leads to widespread upregulation of im- mune and, more specifically, antiviral genes ...of TDP-43 function in the nervous system might ... See full document
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Tdp-43 cryptic exons are highly variable between cell types
... of TDP-43 is ...of TDP-43 deletion to specifically test therapeutic strategies that rescue TDP-43 mechanism of action rather than directly targeting individual cryptic ...of ... See full document
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Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
... monoclonal TDP-43 antibody or antibodies toward pS403/S404-phosphorylated TDP-43, ubiquitin, glial fibrillary acidic protein (GFAP), ionized calcium-bind- ing adaptor molecule 1 (IBA-1), ... See full document
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