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

Abstract Intracellular mislocalization of TAR DNA-binding protein 43 (TDP-43), a nuclear DNA/RNA-binding protein involved in RNA metabolism, is a pathological hallmark of amyotrophic lateral sclerosis (ALS). Although the aggregation-prone, TDP-43 C-terminal domain is widely considered as a key compo...

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Hauptverfasser: Kohei Nishino, Seiji Watanabe, Jin Shijie, Yuri Murata, Kotaro Oiwa, Okiru Komine, Fumito Endo, Hitomi Tsuiji, Manabu Abe, Kenji Sakimura, Amit Mishra, Koji Yamanaka
Format: Artigo
Sprache:Inglês
Veröffentlicht: BMC 2019-07-01
Schriftenreihe:Acta Neuropathologica Communications
Schlagworte:
Amyotrophic lateral sclerosis (ALS)
TAR DNA-binding protein 43 (TDP-43)
Motor dysfunction
TDP-43 knock-in mice
Notch1
Akt
Online Zugang:http://link.springer.com/article/10.1186/s40478-019-0776-5
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