Ontology highlight
ABSTRACT:
SUBMITTER: Ehrnhoefer DE
PROVIDER: S-EPMC5839066 | biostudies-literature | 2018 Mar
REPOSITORIES: biostudies-literature
Ehrnhoefer Dagmar E DE Martin Dale D O DDO Schmidt Mandi E ME Qiu Xiaofan X Ladha Safia S Caron Nicholas S NS Skotte Niels H NH Nguyen Yen T N YTN Vaid Kuljeet K Southwell Amber L AL Engemann Sabine S Franciosi Sonia S Hayden Michael R MR
Acta neuropathologica communications 20180306 1
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 be due to its increased binding to the autophagy adapter p62. This is accompanied by ...[more]