The early pathogenic events leading to neurodegeneration in Huntington disease are not clear. A recent paper shows that mutating a caspase-6 cleavage site in the huntingtin protein is sufficient to prevent pathogenesis.
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Fryer, J., Zoghbi, H. Huntingtin's critical cleavage. Nat Neurosci 9, 1088–1089 (2006). https://doi.org/10.1038/nn0906-1088
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DOI: https://doi.org/10.1038/nn0906-1088