A robust TDP-43 knock-in mouse model of ALS

A robust TDP-43 knock-in mouse model of ALS

Abstract Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset degenerative disorder of motor neurons. The diseased spinal cord motor neurons of more than 95% of amyotrophic lateral sclerosis (ALS) patients are characterized by the mis-metabolism of the RNA/DNA-binding protein TDP-43 (ALS-TDP)...

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Bibliographic Details
Main Authors: Shih-Ling Huang, Lien-Szu Wu, Min Lee, Chin-Wen Chang, Wei-Cheng Cheng, Yu-Sheng Fang, Yun-Ru Chen, Pei-Lin Cheng, Che-Kun James Shen
Format: Article
Language:English
Published: BMC 2020-01-01
Series:Acta Neuropathologica Communications
Subjects:
Amyotrophic lateral sclerosis (ALS)
TAR DNA-binding protein 43 (TDP-43)
Homologous knock-in mouse models with ALS-associated TDP-43 mutations
ALS-TDP pathogenesis
Autonomous spinal cord motor neuron degeneration
Mis-splicing of Bcl-2 pre-mRNA
Online Access:https://doi.org/10.1186/s40478-020-0881-5

Internet

https://doi.org/10.1186/s40478-020-0881-5

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