Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes

James N Sleigh; Andrew P Tosolini; David Gordon; Anny Devoy; Pietro Fratta; Elizabeth M C Fisher; Kevin Talbot; Giampietro Schiavo

doi:10.1016/j.celrep.2020.02.078

Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes

James N Sleigh, Andrew P Tosolini, David Gordon, Anny Devoy, Pietro Fratta, Elizabeth M C Fisher, Kevin Talbot, Giampietro Schiavo

Basic and Clinical Neuroscience

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Abstract

Sleigh et al. address the importance of disturbances in axonal transport in two mouse models for ALS. They find that deficiencies in the in vivo axonal delivery of target tissue-derived survival factors are not a common feature of all ALS mouse models, suggesting mechanistic distinctions in different ALS-linked genes.

Original language	English
Pages (from-to)	3655-3662.e2
Journal	Cell Reports
Volume	30
Issue number	11
Early online date	17 Mar 2020
DOIs	https://doi.org/10.1016/j.celrep.2020.02.078
Publication status	Published - 17 Mar 2020

Keywords

ALS
MND
RNA-binding protein
TARDBP
amyotrophic lateral sclerosis
intravital imaging
motor neuron disease

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10.1016/j.celrep.2020.02.078Licence: CC BY

Mice Carrying ALS Mutant_SLEIGH_Acc19Feb2020_GOLD VoR (CC BY)Final published version, 2.49 MBLicence: CC BY

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AU - Gordon, David

AU - Devoy, Anny

AU - Fratta, Pietro

AU - Fisher, Elizabeth M C

AU - Talbot, Kevin

AU - Schiavo, Giampietro

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KW - MND

KW - RNA-binding protein

KW - TARDBP

KW - amyotrophic lateral sclerosis

KW - intravital imaging

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Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes

Abstract

Keywords

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