A nonsense mutation in mouse Tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.

A nonsense mutation in mouse Tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.

Mutations in TARDBP, encoding Tar DNA binding protein-43 (TDP43), cause amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Attempts to model TDP43 dysfunction in mice have used knockouts or transgenic overexpressors, which have revealed the difficulties of manipulating TDP43, who...

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Bibliographic Details
Main Authors:	Thomas Ricketts, Philip McGoldrick, Pietro Fratta, Hugo M de Oliveira, Rosie Kent, Vinaya Phatak, Sebastian Brandner, Gonzalo Blanco, Linda Greensmith, Abraham Acevedo-Arozena, Elizabeth M C Fisher
Format:	Article
Language:	English
Published:	Public Library of Science (PLoS) 2014-01-01
Series:	PLoS ONE
Online Access:	http://europepmc.org/articles/PMC3897576?pdf=render

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