Article
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.
Acta neuropathologica communications - 25 Jun 2015
Mitchell Jacqueline C, Constable Remy, So Eva, Vance Caroline, Scotter Emma, Glover Leanne, Hortobagyi Tibor, Arnold Eveline S, Ling Shuo-Chien, McAlonis Melissa, Da Cruz Sandrine, Polymenidou Magda, Tessarolo Lino, Cleveland Don W, Shaw Christopher E
Abstract excerpt
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disorder, and cytoplasmic inclusions containing transactive response (TAR) DNA binding protein (TDP-43) are present in ~90 % of cases. Here we report detailed pathology in human TDP-43 transgenic mice that recapitulate key features of TDP-43-linked ALS. RESULTS: Expression of human wild-type TDP-43 (TDP-43(WT))...
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