Article
Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease.
Acta neuropathologica communications - 6 Mar 2018
Ehrnhoefer Dagmar E, Martin Dale D O, Schmidt Mandi E, Qiu Xiaofan, Ladha Safia, Caron Nicholas S, Skotte Niels H, Nguyen Yen T N, Vaid Kuljeet, Southwell Amber L, Engemann Sabine, Franciosi Sonia, Hayden Michael R
Abstract excerpt
Huntington disease (HD) is caused by the expression of mutant huntingtin (mHTT) bearing a polyglutamine expansion. In HD, mHTT accumulation is accompanied by a dysfunction in basal autophagy, which manifests as specific defects in cargo loading during selective autophagy. Here we show that the expression of mHTT resistant to proteolysis at the caspase cleavage site D586 (C6R mHTT) increases autophagy, which may...
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