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Showing 1–5 of 5 results
Advanced filters: Author: "Albert R La Spada" Clear advanced filters

  • Inherited neurodegenerative conditions such as Huntington's disease have proximal causes (a defective gene) and downstream causes (pathological events caused by that gene). Albert R. La Spada examines efforts to target bad genes with gene knockdown approaches on the eve of a clinical trial designed to silence the causative gene in familial amyotrophic lateral sclerosis. Masahisa Katsuno, Hiroaki Adachi and Gen Sobue examine the possibility of targeting a potentially damaging downstream event in Huntington's disease—dysregulated cholesterol metabolism in the brain.

    • Albert R La Spada
    Comments & Opinion
    Nature Medicine
    Volume: 15, P: 252-253

  • Does cell-to-cell spreading of misfolded proteins occur in all neurodegenerative disorders? A study in this issue of Nature Neuroscience now demonstrates propagation of mutant huntingtin in brain slice cultures and in vivo, thereby extending the process of cell-to-cell propagation of misfolded proteins to Huntington's disease.

    • Albert R La Spada
    News & Views
    Nature Neuroscience
    Volume: 17, P: 1014-1015

  • The search for compounds to treat neurodegenerative disorders is especially pressing given the rapidly growing elderly human population and has led to the consideration of sirtuin proteins as potential therapeutic candidates. Two studies now report that modulating the expression of the sirtuin Sirt1 has therapeutic benefit in Huntington's disease mouse models and identify putative downstream targets of Sirt1 involved in improved disease outcomes (pages 159–165 and 153–158).

    • Albert R La Spada
    News & Views
    Nature Medicine
    Volume: 18, P: 24-26

  • Excessive stimulation of glutamate receptors results in excitotoxicity and has a role in a variety of neurodegenerative disorders, including Huntington's disease. By blocking pathological extrasynaptic activity but preserving normal synaptic function, the N-methyl-D-aspartic acid (NMDA) receptor antagonist memantine—at the proper dosage—emerges as a potential treatment for such neurological disorders (pages 1407–1413).

    • Albert R La Spada
    News & Views
    Nature Medicine
    Volume: 15, P: 1355-1356

  • Expansions of repeat sequences cause some of the most common inherited neurological diseases. But how do repeats in DNA lead to pathogenesis? This Review considers the diverse mechanisms that are now emerging, including aberrant splicing, post-translational modification and autophagy.

    • Albert R. La Spada
    • J. Paul Taylor
    Reviews
    Nature Reviews Genetics
    Volume: 11, P: 247-258