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

Kohei Nishino
Kohei Nishino
Seiji Watanabe
Seiji Watanabe
Yuri Murata
Yuri Murata
Kotaro Oiwa
Kotaro Oiwa

Acta Neuropathologica Communications, pp. 1-15, 2019.

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Keywords:
Amyotrophic lateral sclerosis (ALS) TAR DNA-binding protein 43 (TDP-43) Motor dysfunction TDP-43 knock-in mice Notch1More(1+)

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 component of TDP-43 pathology in ALS, recent studies i...More

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