{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:22:06Z","timestamp":1791303726470,"version":"4.1.0"},"reference-count":49,"publisher":"Oxford University Press (OUP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,1,15]]},"DOI":"10.1093\/hmg\/ddu460","type":"journal-article","created":{"date-parts":[[2014,9,10]],"date-time":"2014-09-10T04:39:10Z","timestamp":1410323950000},"page":"450-462","source":"Crossref","is-referenced-by-count":38,"title":["A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease"],"prefix":"10.1093","volume":"24","author":[{"given":"Siddharth","family":"Nath","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lise N.","family":"Munsie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ray","family":"Truant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,9,8]]},"reference":[{"key":"key\n\t\t\t\t20170522140506_DDU460C1","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1016\/j.neuron.2010.06.027","article-title":"Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis","volume":"67","year":"2010","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522140506_DDU460C2","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1038\/ng1219","article-title":"Huntingtin interacts with REST\/NRSF to modulate the transcription of NRSE-controlled neuronal genes","volume":"35","year":"2003","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C3","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1385\/MN:28:3:259","article-title":"Wild-type huntingtin plays a role in brain development and neuronal survival","volume":"28","year":"2003","journal-title":"Mol. Neurobiol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C4","doi-asserted-by":"crossref","first-page":"2789","DOI":"10.1093\/hmg\/9.19.2789","article-title":"Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles","volume":"9","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C5","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.tcb.2009.01.005","article-title":"Huntingtin as an essential integrator of intracellular vesicular trafficking","volume":"19","year":"2009","journal-title":"Trends Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C6","doi-asserted-by":"crossref","first-page":"2124","DOI":"10.1038\/emboj.2008.133","article-title":"Huntingtin phosphorylation acts as a molecular switch for anterograde\/retrograde transport in neurons","volume":"27","year":"2008","journal-title":"EMBO J."},{"key":"key\n\t\t\t\t20170522140506_DDU460C7","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/j.cell.2012.12.029","article-title":"Vesicular glycolysis provides on-board energy for fast axonal transport","volume":"152","year":"2013","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522140506_DDU460C8","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","article-title":"Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules","volume":"118","year":"2004","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522140506_DDU460C9","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1136\/jnnp-2012-304636","article-title":"Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records","volume":"84","year":"2013","journal-title":"J Neurol. Neurosurg. Psychiatry"},{"key":"key\n\t\t\t\t20170522140506_DDU460C10","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's disease chromosomes","volume":"72","author":"The Huntington's Disease Collaborative Research Group","year":"1993","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522140506_DDU460C11","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1016\/j.bbrc.2005.10.061","article-title":"siRNA-mediated inhibition of endogenous Huntington disease gene expression induces an aberrant configuration of the ER network in vitro","volume":"338","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"key\n\t\t\t\t20170522140506_DDU460C12","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1006\/jmbi.2001.4624","article-title":"Comparison of ARM and HEAT protein repeats","volume":"309","year":"2001","journal-title":"J. Mol. Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C13","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1093\/hmg\/ddl440","article-title":"The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis","volume":"16","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C14","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","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C15","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1038\/nchembio.582","article-title":"Kinase inhibitors modulate huntingtin cell localization and toxicity","volume":"7","year":"2011","journal-title":"Nat. Chem. Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C16","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1093\/hmg\/ddr075","article-title":"Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease","volume":"20","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C17","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1038\/35023579","article-title":"Neurodegenerative stimuli induce persistent ADF\/cofilin-actin rods that disrupt distal neurite function","volume":"2","year":"2000","journal-title":"Nat. Cell. Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C18","doi-asserted-by":"crossref","first-page":"C828","DOI":"10.1152\/ajpcell.00066.2006","article-title":"Formation of actin-ADF\/cofilin rods transiently retards decline of mitochondrial potential and ATP in stressed neurons","volume":"291","year":"2006","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C19","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1093\/hmg\/ddg046","article-title":"Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice","volume":"12","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2174\/156652411794474419","article-title":"Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease","volume":"11","year":"2011","journal-title":"Curr. Mol. Med."},{"key":"key\n\t\t\t\t20170522140506_DDU460C21","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1101\/gad.1657108","article-title":"Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging","volume":"22","year":"2008","journal-title":"Genes Dev."},{"key":"key\n\t\t\t\t20170522140506_DDU460C22","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.1128\/MCB.6.5.1767","article-title":"Large changes in intracellular pH and calcium observed during heat shock are not responsible for the induction of heat shock proteins in Drosophila melanogaster","volume":"6","year":"1986","journal-title":"Mol. Cell. Biol."},{"issue":"Pt 23","key":"key\n\t\t\t\t20170522140506_DDU460C23","first-page":"2925","article-title":"HSF1 granules: a novel stress-induced nuclear compartment of human cells","volume":"110","year":"1997","journal-title":"J. Cell. Sci."},{"key":"key\n\t\t\t\t20170522140506_DDU460C24","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/S0076-6879(07)31005-7","article-title":"Mammalian stress granules and processing bodies","volume":"431","year":"2007","journal-title":"Methods Enzymol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C25","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1093\/hmg\/dds554","article-title":"The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal","volume":"22","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522140506_DDU460C26","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1016\/j.bpj.2013.06.030","article-title":"Structure and topology of the huntingtin 1\u201317 membrane anchor by a combined solution and solid-state NMR approach","volume":"105","year":"2013","journal-title":"Biophys J."},{"key":"key\n\t\t\t\t20170522140506_DDU460C27","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1016\/S0955-0674(97)80025-7","article-title":"The diversity of Rab proteins in vesicle transport","volume":"9","year":"1997","journal-title":"Curr. Opin. Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/bj20031000","article-title":"Clathrin-dependent endocytosis","volume":"377","year":"2004","journal-title":"Biochem. J."},{"key":"key\n\t\t\t\t20170522140506_DDU460C29","doi-asserted-by":"crossref","first-page":"28212","DOI":"10.1074\/jbc.M111612200","article-title":"Huntingtin-associated protein 1 interacts with hepatocyte growth factor-regulated tyrosine kinase substrate and functions in endosomal trafficking","volume":"277","year":"2002","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522140506_DDU460C30","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1126\/science.1140621","article-title":"A selective activity-dependent requirement for dynamin 1 in synaptic vesicle endocytosis","volume":"316","year":"2007","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522140506_DDU460C31","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's disease","volume":"172","year":"2006","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C32","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/j.bbagen.2011.09.009","article-title":"The intracellular trafficking pathway of transferrin","volume":"1820","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"issue":"Pt 24","key":"key\n\t\t\t\t20170522140506_DDU460C33","first-page":"4773","article-title":"Biochemical analysis of distinct Rab5- and Rab11-positive endosomes along the transferrin pathway","volume":"112","year":"1999","journal-title":"J. Cell. Sci."},{"key":"key\n\t\t\t\t20170522140506_DDU460C34","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1038\/nrm1315","article-title":"Endocytic recycling","volume":"5","year":"2004","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C35","doi-asserted-by":"crossref","first-page":"12110","DOI":"10.1074\/jbc.M809277200","article-title":"Rab7 regulates late endocytic trafficking downstream of multivesicular body biogenesis and cargo sequestration","volume":"284","year":"2009","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522140506_DDU460C36","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1146\/annurev.biochem.73.011303.074134","article-title":"The mammalian unfolded protein response","volume":"74","year":"2005","journal-title":"Annu. Rev. Biochem."},{"key":"key\n\t\t\t\t20170522140506_DDU460C37","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.mrfmmm.2004.06.056","article-title":"ER stress and the unfolded protein response","volume":"569","year":"2005","journal-title":"Mutat. Res."},{"key":"key\n\t\t\t\t20170522140506_DDU460C38","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1038\/ncb2838","article-title":"Spatial sequestration of misfolded proteins by a dynamic chaperone pathway enhances cellular fitness during stress","volume":"15","year":"2013","journal-title":"Nat. Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C39","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1172\/JCI0216886","article-title":"Orchestrating the unfolded protein response in health and disease","volume":"110","year":"2002","journal-title":"J. Clin. Invest."},{"key":"key\n\t\t\t\t20170522140506_DDU460C40","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/nrm2199","article-title":"Signal integration in the endoplasmic reticulum unfolded protein response","volume":"8","year":"2007","journal-title":"Nat. Rev. Mol. Cell. Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C41","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.bbadis.2009.08.010","article-title":"Cause and consequence: mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases","volume":"1802","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20170522140506_DDU460C42","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1083\/jcb.201003008","article-title":"Actin in dendritic spines: connecting dynamics to function","volume":"189","year":"2010","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522140506_DDU460C43","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1126\/science.1059581","article-title":"Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease","volume":"293","year":"2001","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522140506_DDU460C44","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1002\/anie.201206561","article-title":"Discovery of a novel aggregation domain in the huntingtin protein: implications for the mechanisms of Htt aggregation and toxicity","volume":"52","year":"2013","journal-title":"Angew. Chem. Int Ed. Engl."},{"key":"key\n\t\t\t\t20170522140506_DDU460C45","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1016\/j.str.2009.08.002","article-title":"Secondary structure of Huntingtin amino-terminal region","volume":"17","year":"2009","journal-title":"Structure"},{"key":"key\n\t\t\t\t20170522140506_DDU460C46","doi-asserted-by":"crossref","first-page":"11597","DOI":"10.1021\/jp206373g","article-title":"Secondary structures of native and pathogenic huntingtin N-terminal fragments","volume":"115","year":"2011","journal-title":"J. Phys. Chem. B."},{"key":"key\n\t\t\t\t20170522140506_DDU460C47","doi-asserted-by":"crossref","first-page":"14610","DOI":"10.1073\/pnas.1301342110","article-title":"Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin","volume":"110","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522140506_DDU460C48","doi-asserted-by":"crossref","first-page":"39626","DOI":"10.1074\/jbc.M112.412379","article-title":"Identification of a karyopherin beta1\/beta2 proline-tyrosine nuclear localization signal in huntingtin protein","volume":"287","year":"2012","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522140506_DDU460C49","doi-asserted-by":"crossref","first-page":"2799","DOI":"10.1093\/hmg\/9.19.2799","article-title":"Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells","volume":"9","year":"2000","journal-title":"Hum. Mol. Genet."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","deposited":{"date-parts":[[2019,8,14]],"date-time":"2019-08-14T20:59:47Z","timestamp":1565816387000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddu460"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,9,8]]},"references-count":49,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2014,9,8]]},"published-print":{"date-parts":[[2015,1,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddu460","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,9,8]]}}}