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Chem."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C71","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0076-6879(08)03612-4","article-title":"Monitoring autophagic degradation of p62\/SQSTM1","volume":"452","author":"Bjorkoy","year":"2009","journal-title":"Methods Enzymol."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C72","doi-asserted-by":"crossref","first-page":"2196","DOI":"10.1093\/hmg\/ddn120","article-title":"Premature aging in mice activates a systemic metabolic response involving autophagy induction","volume":"17","author":"Marino","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C73","doi-asserted-by":"crossref","first-page":"542","DOI":"10.4161\/auto.4600","article-title":"How to interpret LC3 immunoblotting","volume":"3","author":"Mizushima","year":"2007","journal-title":"Autophagy"},{"key":"key\n\t\t\t\t20170522164322_DDQ285C74","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/ncb1730","article-title":"Regulation of autophagy by cytoplasmic p53","volume":"10","author":"Tasdemir","year":"2008","journal-title":"Nat. Cell Biol."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C75","doi-asserted-by":"crossref","first-page":"6926","DOI":"10.1523\/JNEUROSCI.0800-08.2008","article-title":"Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease","volume":"28","author":"Boland","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C76","doi-asserted-by":"crossref","first-page":"2600","DOI":"10.1093\/hmg\/ddm217","article-title":"Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity","volume":"16","author":"Atwal","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C77","doi-asserted-by":"crossref","first-page":"14474","DOI":"10.1074\/jbc.M600364200","article-title":"Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1","volume":"281","author":"Shibata","year":"2006","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C78","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1038\/ncb2021","article-title":"The selective autophagy substrate p62 activates the stress responsive transcription factor Nrf2 through inactivation of Keap1","volume":"12","author":"Komatsu","year":"2010","journal-title":"Nat. Cell Biol."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C79","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/j.bbadis.2008.06.010","article-title":"Autophagy in neurodegeneration and development","volume":"1782","author":"Winslow","year":"2008","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20170522164322_DDQ285C80","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1098\/rstb.1999.0451","article-title":"Are there multiple pathways in the pathogenesis of Huntington's disease?","volume":"354","author":"Aronin","year":"1999","journal-title":"Philos. Trans. R. Soc. Lond. B Biol. Sci."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C81","doi-asserted-by":"crossref","first-page":"8506","DOI":"10.1523\/JNEUROSCI.0924-09.2009","article-title":"Inhibition of autophagy induction delays neuronal cell loss caused by dysfunctional ESCRT-III in frontotemporal dementia","volume":"29","author":"Lee","year":"2009","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522164322_DDQ285C82","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","article-title":"Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice","volume":"87","author":"Mangiarini","year":"1996","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522164322_DDQ285C83","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.nbd.2008.10.009","article-title":"CAG repeat lengths > or =335 attenuate the phenotype in the R6\/2 Huntington's disease transgenic mouse","volume":"33","author":"Dragatsis","year":"2009","journal-title":"Neurobiol. 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