TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy |
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| Authors: | Bradley J Turner Dirk Bäumer Nicholas J Parkinson Jakub Scaber Olaf Ansorge Kevin Talbot |
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| Institution: | (1) MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, South Parks Road, OX1 3QX Oxford, UK;(2) Department of Neuropathology, John Radcliffe Hospital, Headley Way, OX3 9DU Oxford, UK;(3) Department of Clinical Neurology, John Radcliffe Hospital, Headley Way, OX3 9DU Oxford, UK |
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| 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). |
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