Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS. (Q38956576)

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scientific article published on 5 October 2015

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English	Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.	scientific article published on 5 October 2015

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26 February 2020

Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS (English)

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amyotrophic lateral sclerosis

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Annika Van Hummel

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Claire H. Stevens

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Glenda Halliday

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Nikolas K. Haass

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Alexander Volkerling

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Alexander Volkerling

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Amadeus Gladbach

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Stefania Ippati

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Julia van der Hoven

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Mian Bi

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Wei S Lee

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Sarah Krüger

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Andre Bongers

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Matthew Kiernan

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5 October 2015

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Acta Neuropathologica

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https://scigraph.springernature.com/pub.10.1007/s00401-015-1486-0

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Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies

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

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Reactive astrocytes secrete lcn2 to promote neuron death

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Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9

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21 January 2018

Neuronal sensitivity to TDP-43 overexpression is dependent on timing of induction

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New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications

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Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

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Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling

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

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Inducible, tightly regulated and non-leaky neuronal gene expression in mice.

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21 January 2018

Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.

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Pathologically proven frontotemporal dementia presenting with severe amnesia

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A subcellular prefractionation protocol for minute amounts of mammalian cell cultures and tissue.

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HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice.

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Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

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C57BL/6J congenic Prp-TDP43A315T mice develop progressive neurodegeneration in the myenteric plexus of the colon without exhibiting key features of ALS.

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Clinicopathological correlates in frontotemporal dementia

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How preserved is episodic memory in behavioral variant frontotemporal dementia?

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In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer's disease.

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21 January 2018

Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats

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21 January 2018

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

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Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.

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21 January 2018

Pronuclear injection for the production of transgenic mice

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Phosphorylated Tau interacts with c-Jun N-terminal kinase-interacting protein 1 (JIP1) in Alzheimer disease

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Staging TDP-43 pathology in Alzheimer's disease

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TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

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Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits

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Sodium selenate mitigates tau pathology, neurodegeneration, and functional deficits in Alzheimer's disease models

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10.1007/S00401-015-1486-0

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