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Mouse with Endogenous SOD1 Mutation: A New Model of Early ALS?
December 17, 2014

For the first time, researchers investigating the role of superoxide dismutase 1 in amyotrophic lateral sclerosis have a rodent model that does not grossly overexpress the gene. Scientists have characterized mice with an ALS-linked mutation in their endogenous SOD1 gene—a substitution...

TDP-43 Structure Reveals Two-Faced Amino End
December 12, 2014
The amino end of TDP-43 has evaded structural characterization because of its penchant for aggregation. Now it has finally revealed its shape. Two shapes, in fact: The domain flips between a well-folded nucleic acid-binding conformation and a loose, aggregation-prone jumble, according to a paper in the Proceedings of the National Academy of Sciences this week. Unexpectedly, the folded conformation looks much like ubiquitin, but this version binds nucleic acids instead of proteins...

Helper Drug Boosts Response to Riluzole in ALS Mice
December 5, 2014
People with amyotrophic lateral sclerosis have one treatment option approved by the Food and Drug Administration: riluzole, which extends life by only a few months. A study in the November 21 Annals of Clinical and Translational Neurology online suggests the drug might be more powerful if it had a pharmacological sidekick. Combining riluzole with a drug called elacridar improved muscle function and lengthened the lifespan of ALS model mice compared with riluzole treatment alone...

Impaired Oxidized mRNA Clearance May Contribute to Neurodegenerative Disease
December 2, 2014
DNA damage likely contributes to neuronal degeneration in diseases such as ALS, but does RNA damage also play a role? A growing body of evidence implicates RNA oxidation as an early contributor to ALS (Chang et. al., 2008)...

Multiple Mutations Hasten ALS Onset, Suggests Genetic Study
November 27, 2014
The genomes of people with amyotrophic lateral sclerosis, even so-called sporadic cases, are often blemished by rare variants that may increase risk for the disease. Some unlucky folk tote two or even three of these potential risk alleles, and they develop the disease a decade earlier...

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