. Large proportion of amyotrophic lateral sclerosis cases in Sardinia due to a single founder mutation of the TARDBP gene. Arch Neurol. 2011 May;68(5):594-8. Epub 2011 Jan 10 PubMed.


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  1. Thank you for this well-written article.

    I would like to add this recent study, which showed that nucleic acid binding activity is required for TDP-43-mediated toxicity in flies.

    C-terminal fragments of TDP-43 lack such activity, but are still capable of forming aggregates. This supports the hypothesis that the aggregates themselves are not toxic, and that instead, the toxicity is related to a modulation of the endogenous function of this nucleic acid binding protein.

    Therefore, studies addressing its DNA and RNA targets may be very important in understanding TDP-43-mediated neurodegeneration.


    . TDP-43-mediated neuron loss in vivo requires RNA-binding activity. PLoS One. 2010;5(8):e12247. PubMed.

    View all comments by Brad Friedman

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