Article
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.
Acta neuropathologica - 1 Nov 2015
Ke Yazi D, van Hummel Annika, Stevens Claire H, Gladbach Amadeus, Ippati Stefania, Bi Mian, Lee Wei S, Krüger Sarah, van der Hoven Julia, Volkerling Alexander, Bongers Andre, Halliday Glenda, Haass Nikolas K, Kiernan Matthew, Delerue Fabien, Ittner Lars M
Abstract excerpt
The nuclear transactive response DNA-binding protein 43 (TDP-43) undergoes relocalization to the cytoplasm with formation of cytoplasmic deposits in neurons in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Pathogenic mutations in the TDP-43-encoding TARDBP gene in familial ALS as well as non-mutant human TDP-43 have been utilized to model FTD/ALS in cell culture and animals,...
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