Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS

Summary: Rare mutations in TARDBP, the gene encoding TDP-43, cause amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is seen in a large majority of ALS patients, suggesting a central pathogenic role of this regulatory protein. The consequences of TARDBP mutations on TDP-43 function and the m...

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Bibliographic Details
Main Authors: Sarah Y. Ebstein, Ilona Yagudayeva, Neil A. Shneider
Format: Article
Language:English
Published: Elsevier 2019-01-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124718319752