{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T20:44:46Z","timestamp":1770842686277,"version":"3.50.1"},"reference-count":35,"publisher":"Frontiers Media SA","funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AG19206"],"award-info":[{"award-number":["AG19206"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS041449"],"award-info":[{"award-number":["NS041449"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS095279"],"award-info":[{"award-number":["NS095279"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS095181"],"award-info":[{"award-number":["NS095181"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Mol. Neurosci."],"DOI":"10.3389\/fnmol.2016.00027","type":"journal-article","created":{"date-parts":[[2016,4,21]],"date-time":"2016-04-21T01:42:57Z","timestamp":1461202977000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":47,"title":["Subcellular Clearance and Accumulation of Huntington Disease Protein: A Mini-Review"],"prefix":"10.3389","volume":"9","author":[{"given":"Ting","family":"Zhao","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yan","family":"Hong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiao-Jiang","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shi-Hua","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2016,4,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"92","DOI":"10.1016\/j.cbpa.2014.05.016","article-title":"Phototransformable fluorescent proteins: future challenges","volume":"20","author":"Adam","year":"2014","journal-title":"Curr. Opin. Chem. Biol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1552","DOI":"10.1126\/science.292.5521.1552","article-title":"Impairment of the ubiquitin-proteasome system by protein aggregation","volume":"292","author":"Bence","year":"2001","journal-title":"Science"},{"key":"B3","doi-asserted-by":"publisher","first-page":"e5128","DOI":"10.1371\/journal.pone.0005128","article-title":"The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6\/2 transgenic mouse model of Huntington\u2019s disease","volume":"4","author":"Bett","year":"2009","journal-title":"PLoS One"},{"key":"B4","doi-asserted-by":"publisher","first-page":"5706","DOI":"10.1073\/pnas.1402215111","article-title":"Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A","volume":"111","author":"Bhat","year":"2014","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B5","doi-asserted-by":"publisher","first-page":"2024","DOI":"10.1038\/nprot.2007.291","article-title":"Tracking intracellular protein movements using photoswitchable fluorescent proteins PS-CFP2 and Dendra2","volume":"2","author":"Chudakov","year":"2007","journal-title":"Nat. Protoc."},{"key":"B6","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1016\/s0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"Davies","year":"1997","journal-title":"Cell"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"B8","doi-asserted-by":"publisher","first-page":"2522","DOI":"10.1093\/hmg\/8.7.1227","article-title":"Nuclear and neuropil aggregates in Huntington\u2019s disease: relationship to neuropathology","volume":"19","author":"Gutekunst","year":"1999","journal-title":"J. Neurosci."},{"key":"B9","doi-asserted-by":"publisher","first-page":"27068","DOI":"10.1074\/jbc.M113.486076","article-title":"Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes","volume":"288","author":"Juenemann","year":"2013","journal-title":"J. Biol. Chem."},{"key":"B11","doi-asserted-by":"publisher","first-page":"146","DOI":"10.1016\/j.tig.2004.01.008","article-title":"Huntingtin-protein interactions and the pathogenesis of Huntington\u2019s disease","volume":"20","author":"Li","year":"2004","journal-title":"Trends Genet."},{"key":"B10","doi-asserted-by":"crossref","first-page":"8473","DOI":"10.1523\/JNEUROSCI.21-21-08473.2001","article-title":"Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington\u2019s disease mice","volume":"21","author":"Li","year":"2001","journal-title":"J. Neurosci."},{"key":"B12","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington\u2019s disease","volume":"10","author":"Lin","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"B13","doi-asserted-by":"publisher","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."},{"key":"B14","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1016\/j.nbd.2003.12.020","article-title":"Proteasome degrades soluble expanded polyglutamine completely and efficiently","volume":"16","author":"Michalik","year":"2004","journal-title":"Neurobiol. Dis."},{"key":"B15","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1172\/JCI45364","article-title":"The importance of integrating basic and clinical research toward the development of new therapies for Huntington\u2019s disease","volume":"121","author":"Munoz-Sanjuan","year":"2011","journal-title":"J. Clin. Invest."},{"key":"B16","doi-asserted-by":"publisher","first-page":"2783","DOI":"10.1523\/JNEUROSCI.0106-08.2008","article-title":"N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking","volume":"28","author":"Orr","year":"2008","journal-title":"J. Neurosci."},{"key":"B17","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1038\/nature05853","article-title":"HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS","volume":"447","author":"Pandey","year":"2007","journal-title":"Nature"},{"key":"B18","doi-asserted-by":"publisher","first-page":"3231","DOI":"10.1093\/hmg\/ddg346","article-title":"Autophagy regulates the processing of amino terminal huntingtin fragments","volume":"12","author":"Qin","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"B19","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1038\/ng1362","article-title":"Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington\u2019s disease","volume":"36","author":"Ravikumar","year":"2004","journal-title":"Nat. Genet."},{"key":"B20","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.bbadis.2011.10.016","article-title":"Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria and selective synaptic degeneration in Huntington\u2019s disease","volume":"1822","author":"Reddy","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"key":"B21","doi-asserted-by":"publisher","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","article-title":"Differential loss of striatal projection neurons in Huntington\u2019s disease","volume":"85","author":"Reiner","year":"1988","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B22","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1002\/ana.410380605","article-title":"Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington\u2019s disease patients","volume":"38","author":"Richfield","year":"1995","journal-title":"Ann. Neurol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1038\/nrneurol.2014.24","article-title":"Huntington disease: natural history, biomarkers and prospects for therapeutics","volume":"10","author":"Ross","year":"2014","journal-title":"Nat. Rev. Neurol."},{"key":"B24","doi-asserted-by":"publisher","first-page":"6168","DOI":"10.1074\/jbc.m114.606293","article-title":"Conophylline protects cells in cellular models of neurodegenerative diseases by inducing mammalian target of rapamycin (mTOR)-independent autophagy","volume":"290","author":"Sasazawa","year":"2015","journal-title":"J. Biol. Chem."},{"key":"B25","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1093\/hmg\/8.3.397","article-title":"Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin","volume":"8","author":"Schilling","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"B26","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1038\/nchembio.250","article-title":"Targeting proteins for degradation","volume":"5","author":"Schrader","year":"2009","journal-title":"Nat. Chem. Biol."},{"key":"B27","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.neuron.2012.05.024","article-title":"Network organization of the huntingtin proteomic interactome in mammalian brain","volume":"75","author":"Shirasaki","year":"2012","journal-title":"Neuron"},{"key":"B28","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1093\/hmg\/ddm320","article-title":"Lysine 63-linked ubiquitination promotes the formation and autophagic clearance of protein inclusions associated with neurodegenerative diseases","volume":"17","author":"Tan","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"B29","doi-asserted-by":"publisher","first-page":"586","DOI":"10.1038\/nchembio.1308","article-title":"Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration","volume":"9","author":"Tsvetkov","year":"2013","journal-title":"Nat. Chem. Biol."},{"key":"B30","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/s1097-2765(04)00151-0","article-title":"Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins","volume":"14","author":"Venkatraman","year":"2004","journal-title":"Mol. Cell"},{"key":"B31","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","article-title":"Huntington disease","volume":"57","author":"Vonsattel","year":"1998","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B33","doi-asserted-by":"publisher","first-page":"1177","DOI":"10.1083\/jcb.200709080","article-title":"Impaired ubiquitin-proteasome system activity in the synapses of Huntington\u2019s disease mice","volume":"180","author":"Wang","year":"2008a","journal-title":"J. Cell Biol."},{"key":"B32","doi-asserted-by":"publisher","first-page":"803","DOI":"10.1083\/jcb.200710158","article-title":"Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin","volume":"181","author":"Wang","year":"2008b","journal-title":"J. Cell Biol."},{"key":"B34","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1093\/hmg\/9.4.503","article-title":"Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice","volume":"9","author":"Wheeler","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"B35","doi-asserted-by":"publisher","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."}],"container-title":["Frontiers in Molecular Neuroscience"],"original-title":[],"deposited":{"date-parts":[[2019,9,6]],"date-time":"2019-09-06T18:24:11Z","timestamp":1567794251000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/Article\/10.3389\/fnmol.2016.00027\/abstract"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,4,21]]},"references-count":35,"URL":"https:\/\/doi.org\/10.3389\/fnmol.2016.00027","relation":{},"ISSN":["1662-5099"],"issn-type":[{"value":"1662-5099","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,4,21]]}}}