{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T08:52:32Z","timestamp":1793609552846,"version":"4.5.0"},"reference-count":106,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2021,12,22]],"date-time":"2021-12-22T00:00:00Z","timestamp":1640131200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100018956","name":"NIHR Cambridge Biomedical Research Centre","doi-asserted-by":"publisher","award":["BRC-1215-20014"],"award-info":[{"award-number":["BRC-1215-20014"]}],"id":[{"id":"10.13039\/501100018956","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"NIHR","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"crossref","award":["203151\/Z\/16\/Z"],"award-info":[{"award-number":["203151\/Z\/16\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,9,14]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Huntington's disease is a neurodegenerative disorder caused by CAG expansions in the huntingtin (HTT) gene. Modelling Huntington's disease is challenging, as rodent and cellular models poorly recapitulate the disease as seen in ageing humans. To address this, we generated induced neurons through direct reprogramming of human skin fibroblasts, which retain age-dependent epigenetic characteristics.<\/jats:p>\n                  <jats:p>Huntington's disease induced neurons (HD-iNs) displayed profound deficits in autophagy, characterized by reduced transport of late autophagic structures from the neurites to the soma. These neurite-specific alterations in autophagy resulted in shorter, thinner and fewer neurites specifically in HD-iNs. CRISPRi-mediated silencing of HTT did not rescue this phenotype but rather resulted in additional autophagy alterations in control induced neurons, highlighting the importance of wild-type HTT in normal neuronal autophagy.<\/jats:p>\n                  <jats:p>In summary, our work identifies a distinct subcellular autophagy impairment in adult patient derived Huntington's disease neurons and provides a new rationale for future development of autophagy activation therapies.<\/jats:p>","DOI":"10.1093\/brain\/awab473","type":"journal-article","created":{"date-parts":[[2021,12,19]],"date-time":"2021-12-19T07:06:31Z","timestamp":1639897591000},"page":"3035-3057","source":"Crossref","is-referenced-by-count":57,"title":["Distinct subcellular autophagy impairments in induced neurons from patients with Huntington's disease"],"prefix":"10.1093","volume":"145","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8281-4785","authenticated-orcid":false,"given":"Karolina","family":"Pircs","sequence":"first","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janelle","family":"Drouin-Ouellet","sequence":"additional","affiliation":[{"name":"Faculty of Pharmacy, Universit\u00e9 de Montr\u00e9al , Montreal, QC H3T 1J4 , Canada"},{"name":"Developmental and Regenerative Neurobiology, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Division of Neurobiology and Lund Stem Cell Center, BMC A11 and B10, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivien","family":"Horv\u00e1th","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeovanis","family":"Gil","sequence":"additional","affiliation":[{"name":"Oncology and Pathology, Kamprad Lab, Department of Clinical Sciences, Lund University , S-221 85 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melinda","family":"Rezeli","sequence":"additional","affiliation":[{"name":"Clinical Protein Science and Imaging, Department of Biomedical Engineering, Lund University , S-221 85 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"Garza","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela A","family":"Grassi","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yogita","family":"Sharma","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"St-Amour","sequence":"additional","affiliation":[{"name":"Axe Neurosciences, Centre de recherche du CHU de Qu\u00e9bec, Universit\u00e9 Laval, CHUL , Qu\u00e9bec, QC, G1E 6W2 , Canada"},{"name":"CERVO Brain Research Center, Universit\u00e9 Laval , Qu\u00e9bec, QC, G1E 1T2 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"Harris","sequence":"additional","affiliation":[{"name":"Wellcome-MRC Cambridge Stem Cell Institute and John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge , Forvie Site, Cambridge CB2 0PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie E","family":"J\u00f6nsson","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pia A","family":"Johansson","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romina","family":"Vuono","sequence":"additional","affiliation":[{"name":"Wellcome-MRC Cambridge Stem Cell Institute and John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge , Forvie Site, Cambridge CB2 0PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaline V","family":"Fazal","sequence":"additional","affiliation":[{"name":"Wellcome-MRC Cambridge Stem Cell Institute and John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge , Forvie Site, Cambridge CB2 0PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Stoker","sequence":"additional","affiliation":[{"name":"Wellcome-MRC Cambridge Stem Cell Institute and John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge , Forvie Site, Cambridge CB2 0PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bob A","family":"Hersbach","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kritika","family":"Sharma","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Lagerwall","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stina","family":"Lagerstr\u00f6m","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petter","family":"Storm","sequence":"additional","affiliation":[{"name":"Developmental and Regenerative Neurobiology, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Division of Neurobiology and Lund Stem Cell Center, BMC A11 and B10, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien S","family":"H\u00e9bert","sequence":"additional","affiliation":[{"name":"Axe Neurosciences, Centre de recherche du CHU de Qu\u00e9bec, Universit\u00e9 Laval, CHUL , Qu\u00e9bec, QC, G1E 6W2 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gy\u00f6rgy","family":"Marko-Varga","sequence":"additional","affiliation":[{"name":"Oncology and Pathology, Kamprad Lab, Department of Clinical Sciences, Lund University , S-221 85 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malin","family":"Parmar","sequence":"additional","affiliation":[{"name":"Developmental and Regenerative Neurobiology, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Division of Neurobiology and Lund Stem Cell Center, BMC A11 and B10, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger A","family":"Barker","sequence":"additional","affiliation":[{"name":"Developmental and Regenerative Neurobiology, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Division of Neurobiology and Lund Stem Cell Center, BMC A11 and B10, Lund University , S-221 84 Lund , Sweden"},{"name":"Wellcome-MRC Cambridge Stem Cell Institute and John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge , Forvie Site, Cambridge CB2 0PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0669-7673","authenticated-orcid":false,"given":"Johan","family":"Jakobsson","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neurogenetics, Department of Experimental Medical Science, Wallenberg Neuroscience Center and Lund Stem Cell Center, BMC A11, Lund University , S-221 84 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,12,22]]},"reference":[{"issue":"6","key":"2022091416352374400_awab473-B1","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes","volume":"72","author":"Macdonald","year":"1993","journal-title":"Cell"},{"issue":"7","key":"2022091416352374400_awab473-B2","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","article-title":"Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington\u2019s disease in the TRACK-HD study: Analysis of 36-month observational data","volume":"12","author":"Tabrizi","year":"2013","journal-title":"Lancet Neurol"},{"issue":"4","key":"2022091416352374400_awab473-B3","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1016\/j.cell.2019.06.036","article-title":"CAG repeat not polyglutamine length determines timing of Huntington\u2019s disease onset","volume":"178","author":"Genetic Modifiers of Huntington\u2019s Disease Consortium. Electronic address ghmhe, Genetic Modifiers of Huntington\u2019s Disease C","year":"2019","journal-title":"Cell"},{"key":"2022091416352374400_awab473-B4","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1002\/ajmg.b.30992","article-title":"CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches","volume":"153B","author":"Langbehn","year":"2010","journal-title":"Am J Med Genet B Neuropsychiatr Genet"},{"issue":"5","key":"2022091416352374400_awab473-B5","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1111\/j.1471-4159.2010.06672.x","article-title":"Differential vulnerability of neurons in Huntington\u2019s disease: The role of cell type-specific features","volume":"113","author":"Han","year":"2010","journal-title":"J Neurochem"},{"issue":"6","key":"2022091416352374400_awab473-B6","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1016\/j.celrep.2018.07.017","article-title":"Huntingtin aggregation impairs autophagy, leading to argonaute-2 accumulation and global microRNA dysregulation","volume":"24","author":"Pircs","year":"2018","journal-title":"Cell Rep."},{"key":"2022091416352374400_awab473-B7","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1080\/15548627.2020.1760014","article-title":"Impact of differential and time-dependent autophagy activation on therapeutic efficacy in a model of Huntington disease","volume":"17","author":"Brattas","year":"2021","journal-title":"Autophagy"},{"issue":"5","key":"2022091416352374400_awab473-B8","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1038\/nn.2528","article-title":"Cargo recognition failure is responsible for inefficient autophagy in Huntington\u2019s disease","volume":"13","author":"Martinez-Vicente","year":"2010","journal-title":"Nat Neurosci"},{"issue":"1","key":"2022091416352374400_awab473-B9","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.tins.2014.09.003","article-title":"Autophagy in Huntington disease and huntingtin in autophagy","volume":"38","author":"Martin","year":"2015","journal-title":"Trends Neurosci"},{"issue":"4","key":"2022091416352374400_awab473-B10","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1523\/JNEUROSCI.1870-13.2014","article-title":"The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation","volume":"34","author":"Wong","year":"2014","journal-title":"J Neurosci"},{"issue":"8","key":"2022091416352374400_awab473-B11","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1038\/nm.3232","article-title":"The role of autophagy in neurodegenerative disease","volume":"19","author":"Nixon","year":"2013","journal-title":"Nat Med"},{"issue":"20","key":"2022091416352374400_awab473-B12","doi-asserted-by":"crossref","first-page":"3702","DOI":"10.2174\/0929867325666180226094351","article-title":"Role of autophagy in Parkinson\u2019s disease","volume":"26","author":"Cerri","year":"2019","journal-title":"Curr Med Chem"},{"issue":"7095","key":"2022091416352374400_awab473-B13","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1038\/nature04724","article-title":"Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice","volume":"441","author":"Hara","year":"2006","journal-title":"Nature"},{"key":"2022091416352374400_awab473-B14","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/nature04723","article-title":"Loss of autophagy in the central nervous system causes neurodegeneration in mice","volume":"441","author":"Komatsu","year":"2006","journal-title":"Nature"},{"issue":"2","key":"2022091416352374400_awab473-B15","doi-asserted-by":"crossref","first-page":"89","DOI":"10.3858\/emm.2012.44.2.031","article-title":"Neuronal autophagy and neurodegenerative diseases","volume":"44","author":"Son","year":"2012","journal-title":"Exp. Mol. Med."},{"issue":"5","key":"2022091416352374400_awab473-B16","doi-asserted-by":"crossref","first-page":"381","DOI":"10.14348\/molcells.2015.0034","article-title":"Autophagy in neurodegenerative diseases: From mechanism to therapeutic approach","volume":"38","author":"Nah","year":"2015","journal-title":"Mol. Cell"},{"key":"2022091416352374400_awab473-B17","doi-asserted-by":"crossref","first-page":"2457","DOI":"10.1007\/s00415-017-8647-0","article-title":"An open-label study to assess the feasibility and tolerability of rilmenidine for the treatment of Huntington\u2019s disease","volume":"264","author":"Underwood","year":"2017","journal-title":"J Neurol"},{"issue":"11","key":"2022091416352374400_awab473-B18","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1016\/S1474-4422(14)70198-X","article-title":"Safety and efficacy of idalopirdine, a 5-HT6 receptor antagonist, in patients with moderate Alzheimer\u2019s disease (LADDER): A randomised, double-blind, placebo-controlled phase 2 trial","volume":"13","author":"Wilkinson","year":"2014","journal-title":"Lancet Neurol"},{"issue":"4","key":"2022091416352374400_awab473-B19","doi-asserted-by":"crossref","first-page":"901","DOI":"10.3390\/ijms20040901","article-title":"Autophagy activator drugs: A new opportunity in neuroprotection from misfolded protein toxicity","volume":"20","author":"Thellung","year":"2019","journal-title":"Int J Mol Sci"},{"issue":"5","key":"2022091416352374400_awab473-B20","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1016\/j.neuron.2017.01.022","article-title":"Autophagy and neurodegeneration: Pathogenic mechanisms and therapeutic opportunities","volume":"93","author":"Menzies","year":"2017","journal-title":"Neuron"},{"issue":"8","key":"2022091416352374400_awab473-B21","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.15252\/emmm.201607471","article-title":"REST suppression mediates neural conversion of adult human fibroblasts via microRNA-dependent and -independent pathways","volume":"9","author":"Drouin-Ouellet","year":"2017","journal-title":"EMBO Mol Med"},{"issue":"6","key":"2022091416352374400_awab473-B22","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/j.stem.2015.09.001","article-title":"Directly reprogrammed human neurons retain aging-associated transcriptomic signatures and reveal age-related nucleocytoplasmic defects","volume":"17","author":"Mertens","year":"2015","journal-title":"Cell Stem Cell"},{"key":"2022091416352374400_awab473-B23","doi-asserted-by":"crossref","first-page":"e18648","DOI":"10.7554\/eLife.18648","article-title":"Maintenance of age in human neurons generated by microRNA-based neuronal conversion of fibroblasts","volume":"5","author":"Huh","year":"2016","journal-title":"Elife"},{"issue":"6","key":"2022091416352374400_awab473-B24","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington\u2019s disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J Neuropathol Exp Neurol"},{"key":"2022091416352374400_awab473-B25","first-page":"56904","article-title":"Simple generation of a high yield culture of induced neurons from human adult skin fibroblasts","volume":"132","author":"Shrigley","year":"2018","journal-title":"J Vis Exp"},{"issue":"5","key":"2022091416352374400_awab473-B26","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1016\/j.cell.2013.02.022","article-title":"Repurposing CRISPR as an RNA-guided platform for sequence-specific control of gene expression","volume":"152","author":"Qi","year":"2013","journal-title":"Cell"},{"issue":"2","key":"2022091416352374400_awab473-B27","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.cell.2013.06.044","article-title":"CRISPR-mediated modular RNA-guided regulation of transcription in eukaryotes","volume":"154","author":"Gilbert","year":"2013","journal-title":"Cell"},{"issue":"1","key":"2022091416352374400_awab473-B28","doi-asserted-by":"crossref","first-page":"e03067","DOI":"10.1016\/j.heliyon.2019.e03067","article-title":"Profiling of lincRNAs in human pluripotent stem cell derived forebrain neural progenitor cells","volume":"6","author":"Grassi","year":"2020","journal-title":"Heliyon"},{"issue":"2","key":"2022091416352374400_awab473-B29","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1007\/s00401-017-1786-7","article-title":"Co-occurrence of mixed proteinopathies in late-stage Huntington\u2019s disease","volume":"135","author":"St-Amour","year":"2018","journal-title":"Acta Neuropathol"},{"issue":"1","key":"2022091416352374400_awab473-B30","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1093\/bioinformatics\/bts635","article-title":"STAR: Ultrafast universal RNA-seq aligner","volume":"29","author":"Dobin","year":"2013","journal-title":"Bioinformatics"},{"issue":"7","key":"2022091416352374400_awab473-B31","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1093\/bioinformatics\/btt656","article-title":"FeatureCounts: An efficient general purpose program for assigning sequence reads to genomic features","volume":"30","author":"Liao","year":"2014","journal-title":"Bioinformatics"},{"issue":"12","key":"2022091416352374400_awab473-B32","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1186\/s13059-014-0550-8","article-title":"Moderated estimation of fold change and dispersion for RNA-seq data with DESeq2","volume":"15","author":"Love","year":"2014","journal-title":"Genome Biol"},{"issue":"D1","key":"2022091416352374400_awab473-B33","doi-asserted-by":"crossref","first-page":"D419","DOI":"10.1093\/nar\/gky1038","article-title":"PANTHER version 14: More genomes, a new PANTHER GO-slim and improvements in enrichment analysis tools","volume":"47","author":"Mi","year":"2019","journal-title":"Nucleic Acids Res"},{"issue":"7","key":"2022091416352374400_awab473-B34","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res"},{"issue":"D1","key":"2022091416352374400_awab473-B35","doi-asserted-by":"crossref","first-page":"D607","DOI":"10.1093\/nar\/gky1131","article-title":"STRING v11: Protein-protein association networks with increased coverage, supporting functional discovery in genome-wide experimental datasets","volume":"47","author":"Szklarczyk","year":"2019","journal-title":"Nucleic Acids Res"},{"issue":"10","key":"2022091416352374400_awab473-B36","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1093\/bioinformatics\/btu049","article-title":"Minfi: A flexible and comprehensive bioconductor package for the analysis of infinium DNA methylation microarrays","volume":"30","author":"Aryee","year":"2014","journal-title":"Bioinformatics"},{"issue":"7","key":"2022091416352374400_awab473-B37","doi-asserted-by":"crossref","first-page":"e90","DOI":"10.1093\/nar\/gkt090","article-title":"Low-level processing of Illumina Infinium DNA methylation beadarrays","volume":"41","author":"Triche","year":"2013","journal-title":"Nucleic Acids Res"},{"issue":"D1","key":"2022091416352374400_awab473-B38","doi-asserted-by":"crossref","first-page":"D442","DOI":"10.1093\/nar\/gky1106","article-title":"The PRIDE database and related tools and resources in 2019: Improving support for quantification data","volume":"47","author":"Perez-Riverol","year":"2019","journal-title":"Nucleic Acids Res"},{"key":"2022091416352374400_awab473-B39","doi-asserted-by":"crossref","first-page":"530","DOI":"10.3389\/fnins.2017.00530","article-title":"Direct neuronal reprogramming for disease modeling studies using patient-derived neurons: What have we learned?","volume":"11","author":"Drouin-Ouellet","year":"2017","journal-title":"Front Neurosci"},{"key":"2022091416352374400_awab473-B40","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1038\/s41593-018-0075-7","article-title":"Striatal neurons directly converted from Huntington\u2019s disease patient fibroblasts recapitulate age-associated disease phenotypes","volume":"21","author":"Victor","year":"2018","journal-title":"Nat Neurosci"},{"key":"2022091416352374400_awab473-B41","doi-asserted-by":"crossref","first-page":"2075","DOI":"10.1007\/s12035-020-02258-w","article-title":"Directly reprogrammed neurons express MAPT and APP splice variants pertinent to ageing and neurodegeneration","volume":"58","author":"Habekost","year":"2021","journal-title":"Mol Neurobiol"},{"key":"2022091416352374400_awab473-B42","doi-asserted-by":"crossref","first-page":"e41356","DOI":"10.7554\/eLife.41356","article-title":"Chemical modulation of transcriptionally enriched signaling pathways to optimize the conversion of fibroblasts into neurons","volume":"8","author":"Herdy","year":"2019","journal-title":"Elife"},{"key":"2022091416352374400_awab473-B43","doi-asserted-by":"crossref","first-page":"359","DOI":"10.3389\/fnmol.2017.00359","article-title":"Direct reprogramming rather than iPSC-based reprogramming maintains aging hallmarks in human motor neurons","volume":"10","author":"Tang","year":"2017","journal-title":"Front Mol Neurosci"},{"issue":"10","key":"2022091416352374400_awab473-B44","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1186\/gb-2013-14-10-r115","article-title":"DNA methylation age of human tissues and cell types","volume":"14","author":"Horvath","year":"2013","journal-title":"Genome Biol"},{"issue":"7","key":"2022091416352374400_awab473-B45","doi-asserted-by":"crossref","first-page":"1485","DOI":"10.18632\/aging.101005","article-title":"Huntington\u2019s disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels","volume":"8","author":"Horvath","year":"2016","journal-title":"Aging"},{"issue":"27","key":"2022091416352374400_awab473-B46","doi-asserted-by":"crossref","first-page":"15989","DOI":"10.1073\/pnas.2002144117","article-title":"Cyclic GMP-AMP synthase promotes the inflammatory and autophagy responses in Huntington disease","volume":"117","author":"Sharma","year":"2020","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"1","key":"2022091416352374400_awab473-B47","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1038\/s41467-021-21637-y","article-title":"Mutant Huntingtin stalls ribosomes and represses protein synthesis in a cellular model of Huntington disease","volume":"12","author":"Eshraghi","year":"2021","journal-title":"Nat Commun"},{"issue":"20","key":"2022091416352374400_awab473-B48","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":"2022091416352374400_awab473-B49","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/nature22078","article-title":"Polyglutamine tracts regulate beclin 1-dependent autophagy","volume":"545","author":"Ashkenazi","year":"2017","journal-title":"Nature"},{"issue":"8","key":"2022091416352374400_awab473-B50","doi-asserted-by":"crossref","first-page":"e44214","DOI":"10.1371\/journal.pone.0044214","article-title":"Advantages and limitations of different p62-based assays for estimating autophagic activity in Drosophila","volume":"7","author":"Pircs","year":"2012","journal-title":"PLoS ONE"},{"issue":"1","key":"2022091416352374400_awab473-B51","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1080\/15548627.2015.1100356","article-title":"Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)","volume":"12","author":"Klionsky","year":"2016","journal-title":"Autophagy"},{"issue":"1","key":"2022091416352374400_awab473-B52","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.devcel.2014.06.001","article-title":"Autophagosome biogenesis in primary neurons follows an ordered and spatially regulated pathway","volume":"30","author":"Maday","year":"2014","journal-title":"Dev Cell"},{"issue":"1","key":"2022091416352374400_awab473-B53","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.celrep.2019.06.013","article-title":"Neuronal soma-derived degradative lysosomes are continuously delivered to distal axons to maintain local degradation capacity","volume":"28","author":"Farfel-Becker","year":"2019","journal-title":"Cell Rep"},{"issue":"4","key":"2022091416352374400_awab473-B54","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1083\/jcb.200509091","article-title":"Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington\u2019s disease","volume":"172","author":"Pal","year":"2006","journal-title":"J Cell Biol"},{"issue":"10","key":"2022091416352374400_awab473-B55","doi-asserted-by":"crossref","first-page":"1649","DOI":"10.1242\/jcs.025726","article-title":"Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease","volume":"121","author":"Ravikumar","year":"2008","journal-title":"J Cell Sci"},{"issue":"2","key":"2022091416352374400_awab473-B56","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.nbd.2009.08.003","article-title":"Disruption of Rab11 activity in a knock-in mouse model of Huntington\u2019s disease","volume":"36","author":"Li","year":"2009","journal-title":"Neurobiol Dis"},{"issue":"5","key":"2022091416352374400_awab473-B57","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1007\/s10571-010-9507-y","article-title":"The evolutionarily conserved interaction between LC3 and p62 selectively mediates autophagy-dependent degradation of mutant huntingtin","volume":"30","author":"Tung","year":"2010","journal-title":"Cell Mol Neurobiol"},{"issue":"4","key":"2022091416352374400_awab473-B58","doi-asserted-by":"crossref","first-page":"315","DOI":"10.4161\/auto.2974","article-title":"Autophagy and neurodegeneration","volume":"2","author":"Komatsu","year":"2006","journal-title":"Autophagy"},{"issue":"47","key":"2022091416352374400_awab473-B59","doi-asserted-by":"crossref","first-page":"16889","DOI":"10.1073\/pnas.1420103111","article-title":"Potential function for the Huntingtin protein as a scaffold for selective autophagy","volume":"111","author":"Ochaba","year":"2014","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"3","key":"2022091416352374400_awab473-B60","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1038\/ncb3101","article-title":"Huntingtin functions as a scaffold for selective macroautophagy","volume":"17","author":"Rui","year":"2015","journal-title":"Nat Cell Biol"},{"issue":"1","key":"2022091416352374400_awab473-B61","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1038\/s41598-017-01510-z","article-title":"The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington\u2019s disease patients","volume":"7","author":"Neueder","year":"2017","journal-title":"Sci Rep"},{"issue":"12","key":"2022091416352374400_awab473-B62","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1002\/jnr.24493","article-title":"Phenotype onset in Huntington\u2019s disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene","volume":"97","author":"Franich","year":"2019","journal-title":"J Neurosci Res"},{"issue":"3","key":"2022091416352374400_awab473-B63","doi-asserted-by":"crossref","first-page":"1941","DOI":"10.1111\/ejn.12957","article-title":"Altered lysosomal positioning affects lysosomal functions in a cellular model of Huntington\u2019s disease","volume":"42","author":"Erie","year":"2015","journal-title":"Eur J Neurosci"},{"issue":"19","key":"2022091416352374400_awab473-B64","doi-asserted-by":"crossref","first-page":"7268","DOI":"10.1523\/JNEUROSCI.20-19-07268.2000","article-title":"Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy","volume":"20","author":"Kegel","year":"2000","journal-title":"J Neurosci"},{"issue":"10","key":"2022091416352374400_awab473-B65","doi-asserted-by":"crossref","first-page":"e109621","DOI":"10.1371\/journal.pone.0109621","article-title":"Direct reprogramming of Huntington\u2019s disease patient fibroblasts into neuron-like cells leads to abnormal neurite outgrowth, increased cell death, and aggregate formation","volume":"9","author":"Liu","year":"2014","journal-title":"PLoS One"},{"issue":"12","key":"2022091416352374400_awab473-B66","doi-asserted-by":"crossref","first-page":"3877","DOI":"10.1523\/JNEUROSCI.11-12-03877.1991","article-title":"Proliferative and degenerative changes in striatal spiny neurons in Huntington\u2019s disease: A combined study using the section-Golgi method and calbindin D28k immunocytochemistry","volume":"11","author":"Ferrante","year":"1991","journal-title":"J Neurosci"},{"issue":"22","key":"2022091416352374400_awab473-B67","doi-asserted-by":"crossref","first-page":"6019","DOI":"10.1523\/JNEUROSCI.1251-06.2006","article-title":"Regulation of intracellular trafficking of huntingtin-associated protein-1 is critical for TrkA protein levels and neurite outgrowth","volume":"26","author":"Rong","year":"2006","journal-title":"J Neurosci"},{"issue":"7","key":"2022091416352374400_awab473-B68","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1002\/jnr.23733","article-title":"Autophagy inhibition in endogenous and nutrient-deprived conditions reduces dorsal root ganglia neuron survival and neurite growth in vitro","volume":"94","author":"Clarke","year":"2016","journal-title":"J Neurosci Res"},{"key":"2022091416352374400_awab473-B69","doi-asserted-by":"crossref","first-page":"1514","DOI":"10.3390\/cells9061514","article-title":"Huntington\u2019s Disease\u2014An Outlook on the Interplay of the HTT Protein, Microtubules and Actin Cytoskeletal Components","volume":"9","author":"Taran","year":"2020","journal-title":"Cells"},{"issue":"10","key":"2022091416352374400_awab473-B70","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1038\/nrn3570","article-title":"Choosing an animal model for the study of Huntington\u2019s disease","volume":"14","author":"Pouladi","year":"2013","journal-title":"Nat Rev Neurosci"},{"issue":"6","key":"2022091416352374400_awab473-B71","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.bbadis.2009.04.001","article-title":"Mouse models of Huntington\u2019s disease and methodological considerations for therapeutic trials","volume":"1792","author":"Ferrante","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"2022091416352374400_awab473-B72","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1007\/978-1-4939-7825-0_6","article-title":"Mouse models of Huntington\u2019s disease","volume":"1780","author":"Farshim","year":"2018","journal-title":"Methods Mol Biol"},{"key":"2022091416352374400_awab473-B73","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1007\/978-1-4939-7825-0_10","article-title":"Murine models of Huntington\u2019s disease for evaluating therapeutics","volume":"1780","author":"Kosior","year":"2018","journal-title":"Methods Mol Biol"},{"key":"2022091416352374400_awab473-B74","doi-asserted-by":"crossref","first-page":"e4f8606b742ef3","DOI":"10.1371\/4f8606b742ef3","article-title":"The prevalence of juvenile Huntington\u2019s disease: A review of the literature and meta-analysis","volume":"4","author":"Quarrell","year":"2012","journal-title":"PLoS Curr"},{"issue":"6","key":"2022091416352374400_awab473-B75","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1093\/hmg\/ddg075","article-title":"Transgenic rat model of Huntington\u2019s disease","volume":"12","author":"von Horsten","year":"2003","journal-title":"Hum Mol Genet"},{"issue":"2","key":"2022091416352374400_awab473-B76","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/j.stem.2012.04.027","article-title":"Induced pluripotent stem cells from patients with Huntington\u2019s disease show CAG-repeat-expansion-associated phenotypes","volume":"11","author":"Consortium","year":"2012","journal-title":"Cell Stem Cell"},{"issue":"5","key":"2022091416352374400_awab473-B77","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1038\/nn.4532","article-title":"Developmental alterations in Huntington\u2019s disease neural cells and pharmacological rescue in cells and mice","volume":"20","author":"Consortium","year":"2017","journal-title":"Nat Neurosci"},{"key":"2022091416352374400_awab473-B78","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.brainres.2015.10.001","article-title":"Modeling Huntington\u2019s disease with patient-derived neurons","volume":"1656","author":"Mattis","year":"2017","journal-title":"Brain Res"},{"issue":"4","key":"2022091416352374400_awab473-B79","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1016\/j.celrep.2018.09.076","article-title":"Human Huntington\u2019s disease iPSC-derived cortical neurons display altered transcriptomics, morphology, and maturation","volume":"25","author":"Mehta","year":"2018","journal-title":"Cell Rep"},{"key":"2022091416352374400_awab473-B80","doi-asserted-by":"crossref","first-page":"7","DOI":"10.3389\/fgeed.2020.00007","article-title":"The iNs and outs of direct reprogramming to induced neurons review","volume":"2","author":"Carter","year":"2020","journal-title":"Front. Genome Edit"},{"key":"2022091416352374400_awab473-B81","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1146\/annurev-genet-120417-031534","article-title":"Aging in a dish: iPSC-derived and directly induced neurons for studying brain aging and age-related neurodegenerative diseases","volume":"52","author":"Mertens","year":"2018","journal-title":"Annu Rev Genet."},{"issue":"6","key":"2022091416352374400_awab473-B82","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.stem.2013.11.006","article-title":"Human iPSC-based modeling of late-onset disease via progerin-induced aging","volume":"13","author":"Miller","year":"2013","journal-title":"Cell Stem Cell"},{"issue":"7","key":"2022091416352374400_awab473-B83","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1016\/j.drudis.2014.03.016","article-title":"Transcriptional dysregulation in Huntington\u2019s disease: A failure of adaptive transcriptional homeostasis","volume":"19","author":"Kumar","year":"2014","journal-title":"Drug Discov Today"},{"issue":"5","key":"2022091416352374400_awab473-B84","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1091\/mbc.e08-07-0726","article-title":"Mutant huntingtin impairs post-Golgi trafficking to lysosomes by delocalizing optineurin\/Rab8 complex from the Golgi apparatus","volume":"20","author":"del Toro","year":"2009","journal-title":"Mol Biol Cell"},{"issue":"2","key":"2022091416352374400_awab473-B85","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.neuron.2014.12.019","article-title":"Reinstating aberrant mTORC1 activity in Huntington\u2019s disease mice improves disease phenotypes","volume":"85","author":"Lee","year":"2015","journal-title":"Neuron"},{"issue":"4","key":"2022091416352374400_awab473-B86","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1093\/hmg\/ddu522","article-title":"Depletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington\u2019s model mice","volume":"24","author":"Kurosawa","year":"2015","journal-title":"Hum Mol Genet"},{"issue":"9","key":"2022091416352374400_awab473-B87","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/j.tibs.2006.06.009","article-title":"Huntington\u2019s disease: Seeing the pathogenic process through a genetic lens","volume":"31","author":"Gusella","year":"2006","journal-title":"Trends Biochem Sci"},{"issue":"5","key":"2022091416352374400_awab473-B88","doi-asserted-by":"crossref","first-page":"663","DOI":"10.4161\/auto.6.5.12336","article-title":"Clearance of mutant huntingtin","volume":"6","author":"Li","year":"2010","journal-title":"Autophagy"},{"issue":"349","key":"2022091416352374400_awab473-B89","doi-asserted-by":"crossref","first-page":"ra103","DOI":"10.1126\/scisignal.2005633","article-title":"Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington\u2019s disease","volume":"7","author":"Pryor","year":"2014","journal-title":"Sci Signal"},{"key":"2022091416352374400_awab473-B90","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.ebiom.2016.10.034","article-title":"Therapeutic targeting of autophagy","volume":"14","author":"Towers","year":"2016","journal-title":"EBioMedicine"},{"issue":"4","key":"2022091416352374400_awab473-B91","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.tins.2016.02.002","article-title":"The autophagy-lysosomal pathway in neurodegeneration: A TFEB perspective","volume":"39","author":"Martini-Stoica","year":"2016","journal-title":"Trends Neurosci"},{"issue":"2","key":"2022091416352374400_awab473-B92","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1016\/j.molcel.2008.03.003","article-title":"AMPK phosphorylation of raptor mediates a metabolic checkpoint","volume":"30","author":"Gwinn","year":"2008","journal-title":"Mol Cell"},{"issue":"5","key":"2022091416352374400_awab473-B93","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/j.cell.2006.06.055","article-title":"TSC2 integrates Wnt and energy signals via a coordinated phosphorylation by AMPK and GSK3 to regulate cell growth","volume":"126","author":"Inoki","year":"2006","journal-title":"Cell"},{"issue":"5","key":"2022091416352374400_awab473-B94","doi-asserted-by":"crossref","first-page":"1064","DOI":"10.1016\/j.cell.2013.04.055","article-title":"The eEF2 kinase confers resistance to nutrient deprivation by blocking translation elongation","volume":"153","author":"Leprivier","year":"2013","journal-title":"Cell"},{"issue":"6","key":"2022091416352374400_awab473-B95","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1093\/hmg\/ddv513","article-title":"AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington\u2019s disease","volume":"25","author":"Vazquez-Manrique","year":"2016","journal-title":"Hum Mol Genet"},{"issue":"4","key":"2022091416352374400_awab473-B96","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1007\/s12017-016-8412-z","article-title":"Metformin protects cells from mutant huntingtin toxicity through activation of AMPK and modulation of mitochondrial dynamics","volume":"18","author":"Jin","year":"2016","journal-title":"Neuromolecular Med"},{"issue":"2","key":"2022091416352374400_awab473-B97","doi-asserted-by":"crossref","first-page":"195","DOI":"10.3233\/BPL-180066","article-title":"Crosstalk between MicroRNAs and autophagy in adult neurogenesis: Implications for neurodegenerative disorders","volume":"3","author":"Pircs","year":"2018","journal-title":"Brain Plast"},{"key":"2022091416352374400_awab473-B98","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.15252\/embj.201695235","article-title":"Let-7 regulates radial migration of new-born neurons through positive regulation of autophagy","volume":"36","author":"Petri","year":"2017","journal-title":"EMBO Journal"},{"key":"2022091416352374400_awab473-B99","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1146\/annurev-neuro-071013-014149","article-title":"Autophagy and its normal and pathogenic states in the brain","volume":"37","author":"Yamamoto","year":"2014","journal-title":"Annu Rev Neurosci"},{"issue":"33","key":"2022091416352374400_awab473-B100","doi-asserted-by":"crossref","first-page":"10371","DOI":"10.1523\/JNEUROSCI.1592-09.2009","article-title":"Alterations in cortical excitation and inhibition in genetic mouse models of Huntington\u2019s disease","volume":"29","author":"Cummings","year":"2009","journal-title":"J Neurosci"},{"issue":"10","key":"2022091416352374400_awab473-B101","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1101\/gad.5.10.1777","article-title":"The carboxy-terminal catalytic domain of the GTPase-activating protein inhibits nuclear signal transduction and morphological transformation mediated by the CSF-1 receptor","volume":"5","author":"Bortner","year":"1991","journal-title":"Genes Dev"},{"issue":"6","key":"2022091416352374400_awab473-B102","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1038\/aps.2012.14","article-title":"The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1","volume":"33","author":"Wu","year":"2012","journal-title":"Acta Pharmacol Sin"},{"issue":"11","key":"2022091416352374400_awab473-B103","doi-asserted-by":"crossref","first-page":"2144","DOI":"10.1093\/hmg\/ddq093","article-title":"Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington\u2019s disease","volume":"19","author":"Rose","year":"2010","journal-title":"Hum Mol Genet"},{"issue":"7","key":"2022091416352374400_awab473-B104","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1016\/j.drudis.2014.04.013","article-title":"Huntington\u2019s disease: From disease mechanisms to therapies","volume":"19","author":"Blackstone","year":"2014","journal-title":"Drug Discov Today"},{"issue":"6","key":"2022091416352374400_awab473-B105","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1038\/d41573-021-00088-6","article-title":"Double setback for ASO trials in Huntington disease","volume":"20","author":"Kingwell","year":"2021","journal-title":"Nat Rev Drug Discov"},{"key":"2022091416352374400_awab473-B106","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1038\/d41586-021-01177-7","article-title":"Failure of genetic therapies for Huntington\u2019s devastates community","volume":"593","author":"Kwon","year":"2021","journal-title":"Nature"}],"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/brain\/advance-article-pdf\/doi\/10.1093\/brain\/awab473\/45110470\/awab473.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/145\/9\/3035\/45842507\/awab473.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/145\/9\/3035\/45842507\/awab473.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,14]],"date-time":"2023-11-14T08:22:20Z","timestamp":1699950140000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/article\/145\/9\/3035\/6479694"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,22]]},"references-count":106,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2021,12,22]]},"published-print":{"date-parts":[[2022,9,14]]}},"URL":"https:\/\/doi.org\/10.1093\/brain\/awab473","relation":{},"ISSN":["0006-8950","1460-2156"],"issn-type":[{"value":"0006-8950","type":"print"},{"value":"1460-2156","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,9,1]]},"published":{"date-parts":[[2021,12,22]]}}}