Journal article

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy

Bradley J Turner, Dirk Baumer, Nicholas J Parkinson, Jakub Scaber, Olaf Ansorge, Kevin Talbot

BMC NEUROSCIENCE | BMC | Published : 2008

Abstract

BACKGROUND: Redistribution of nuclear TAR DNA binding protein 43 (TDP-43) to the cytoplasm and ubiquitinated inclusions of spinal motor neurons and glial cells is characteristic of amyotrophic lateral sclerosis (ALS) pathology. Recent evidence suggests that TDP-43 pathology is common to sporadic ALS and familial ALS without SOD1 mutation, but not SOD1-related fALS cases. Furthermore, it remains unclear whether TDP-43 abnormalities occur in non-ALS forms of motor neuron disease. Here, we characterise TDP-43 localisation, expression levels and post-translational modifications in mouse models of ALS and spinal muscular atrophy (SMA). RESULTS: TDP-43 mislocalisation to ubiquitinated inclusions o..

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