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 F. Gordon, Anny Devoy, Pietro Fratta, Elizabeth Fisher, Kevin Talbot, Giampietro Schiavo
Abstract
mice. Deficiencies in retrograde axonal transport of signaling endosomes are therefore not shared by all ALS-linked genes, indicating that there are mechanistic distinctions in the pathogenesis of ALS caused by mutations in different RNA processing genes.
Topics & Concepts
Axoplasmic transportEndosomeAmyotrophic lateral sclerosisBiologyTARDBPNeuroscienceCell biologyMutantMotor neuronNeurodegenerationMutationSOD1GeneGeneticsPathologyMedicineDiseaseSpinal cordIntracellularAmyotrophic Lateral Sclerosis ResearchNeurogenetic and Muscular Disorders ResearchPrion Diseases and Protein Misfolding