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Title: Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice
Authors: Herdewyn, Sarah
Cirillo, Carla
Van Den Bosch, Ludo
Robberecht, Wim
Vanden Berghe, Pieter #
Van Damme, Philip # ×
Issue Date: 2014
Series Title: Molecular neurodegeneration vol:9 issue:1 pages:24
Article number: 10.1186/1750-1326-9-24
Abstract: Intraneuronal inclusions of TAR DNA-binding protein 43 (TDP-43) have been found in the majority of Amyotrophic Lateral Sclerosis (ALS) patients. Mutations in the gene encoding TDP-43 cause familial ALS. Transgenic mice expressing mutant TDP-43 with one such mutation (TDP-43 (A315T)) under control of the murine prion promoter develop motor symptoms, but their use is currently hampered by sudden death. We aimed to understand and overcome the cause of sudden death in TDP-43 (A315T) mice. Since intestinal obstruction was suspected to be the cause, intestinal motility of TDP-43 (A315T) mice was studied in an ex-vivo pellet propulsion assay. The effect on the enteric and motor phenotype was assessed, both in animals on normal chow or on a jellified fiber deprived diet, aimed at preventing intestinal obstruction.
URI: 
ISSN: 1750-1326
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory for Neurobiology (Vesalius Research Center)
Translational Research in GastroIntestinal Disorders
× corresponding author
# (joint) last author

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