{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T19:19:42Z","timestamp":1791314382933,"version":"4.3.1"},"reference-count":55,"publisher":"Oxford University Press (OUP)","issue":"21","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,11,1]]},"DOI":"10.1093\/hmg\/ddm217","type":"journal-article","created":{"date-parts":[[2007,8,18]],"date-time":"2007-08-18T20:13:53Z","timestamp":1187468033000},"page":"2600-2615","source":"Crossref","is-referenced-by-count":348,"title":["Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity"],"prefix":"10.1093","volume":"16","author":[{"given":"Randy Singh","family":"Atwal","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianrun","family":"Xia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah","family":"Pinchev","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jillian","family":"Taylor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard M.","family":"Epand","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":[[2007,8,8]]},"reference":[{"key":"key\n\t\t\t\t20170522131404_DDM217C1","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S1474-4422(04)00737-9","article-title":"Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis","volume":"3","author":"Schols","year":"2004","journal-title":"Lancet Neurol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C2","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington's disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C3","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1038\/ng0595-3","article-title":"Gain of glutamines, gain of function?","volume":"10","author":"Housman","year":"1995","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C4","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1055\/s-2008-1040853","article-title":"Recent insights into the molecular pathogenesis of Huntington disease","volume":"19","author":"Leavitt","year":"1999","journal-title":"Semin. Neurol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C5","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1086\/318207","article-title":"Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo","volume":"68","author":"Leavitt","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C6","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","author":"Gauthier","year":"2004","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522131404_DDM217C7","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/S0896-6273(03)00594-4","article-title":"Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila","volume":"40","author":"Gunawardena","year":"2003","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522131404_DDM217C8","doi-asserted-by":"crossref","first-page":"3224","DOI":"10.1073\/pnas.0400243101","article-title":"Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease","volume":"101","author":"Lee","year":"2004","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522131404_DDM217C9","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","author":"Pal","year":"2006","journal-title":"J. Cell. Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C10","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","author":"Hilditch-Maguire","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C11","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","author":"Reiner","year":"2003","journal-title":"Mol. Neurobiol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C12","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1093\/hmg\/5.8.1093","article-title":"Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation","volume":"5","author":"De Rooij","year":"1996","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C13","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1016\/0896-6273(95)90346-1","article-title":"Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons","volume":"14","author":"DiFiglia","year":"1995","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522131404_DDM217C14","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1093\/hmg\/ddg156","article-title":"Huntingtin contains a highly conserved nuclear export signal","volume":"12","author":"Xia","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C15","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1111\/j.1471-4159.2005.03620.x","article-title":"Interaction of huntingtin fragments with brain membranes\u2013clues to early dysfunction in Huntington's disease","volume":"96","author":"Suopanki","year":"2006","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C16","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","author":"Zuccato","year":"2001","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522131404_DDM217C17","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","author":"Zuccato","year":"2003","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C18","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1126\/science.1092194","article-title":"SUMO modification of Huntingtin and Huntington's disease pathology","volume":"304","author":"Steffan","year":"2004","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522131404_DDM217C19","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","author":"Trettel","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C20","doi-asserted-by":"crossref","first-page":"51S","DOI":"10.1042\/bst025051s","article-title":"Monoclonal antibodies from three new regions of huntingtin, the Huntington's disease protein","volume":"25","author":"Wilkinson","year":"1997","journal-title":"Biochem. Soc. Trans."},{"key":"key\n\t\t\t\t20170522131404_DDM217C21","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1242\/jcs.02856","article-title":"Beyond lectins: the calnexin\/calreticulin chaperone system of the endoplasmic reticulum","volume":"119","author":"Williams","year":"2006","journal-title":"J. Cell Sci."},{"key":"key\n\t\t\t\t20170522131404_DDM217C22","doi-asserted-by":"crossref","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":"key\n\t\t\t\t20170522131404_DDM217C23","doi-asserted-by":"crossref","first-page":"8130","DOI":"10.1074\/jbc.M111289200","article-title":"An amino-terminal amphipathic alpha-helix mediates membrane association of the hepatitis C virus nonstructural protein 5A","volume":"277","author":"Brass","year":"2002","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C24","doi-asserted-by":"crossref","first-page":"240","DOI":"10.2144\/04362BI01","article-title":"Computer-assisted image analysis protocol that quantitatively measures subnuclear protein organization in cell populations","volume":"36","author":"Voss","year":"2004","journal-title":"Biotechniques"},{"key":"key\n\t\t\t\t20170522131404_DDM217C25","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1038\/ng1095-115","article-title":"HEAT repeats in the Huntington's disease protein","volume":"11","author":"Andrade","year":"1995","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C26","doi-asserted-by":"crossref","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":"key\n\t\t\t\t20170522131404_DDM217C27","doi-asserted-by":"crossref","first-page":"80","DOI":"10.2144\/02321st04","article-title":"Live-cell nucleocytoplasmic protein shuttle assay utilizing laser confocal microscopy and FRAP","volume":"32","author":"Howell","year":"2002","journal-title":"Biotechniques"},{"key":"key\n\t\t\t\t20170522131404_DDM217C28","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1083\/jcb.152.1.111","article-title":"Differential localization of Rho GTPases in live cells: regulation by hypervariable regions and RhoGDI binding","volume":"152","author":"Michaelson","year":"2001","journal-title":"J. Cell. Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C29","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1093\/oxfordjournals.jbchem.a002914","article-title":"Dynamics of intracellular granules with CD63-GFP in rat basophilic leukemia cells","volume":"129","author":"Amano","year":"2001","journal-title":"J. Biochem. (Tokyo)"},{"key":"key\n\t\t\t\t20170522131404_DDM217C30","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1083\/jcb.200109030","article-title":"Visualization of Rab9-mediated vesicle transport from endosomes to the trans-Golgi in living cells","volume":"156","author":"Barbero","year":"2002","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C31","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1091\/mbc.11.2.467","article-title":"Rab7: a key to lysosome biogenesis","volume":"11","author":"Bucci","year":"2000","journal-title":"Mol. Biol. Cell"},{"key":"key\n\t\t\t\t20170522131404_DDM217C32","doi-asserted-by":"crossref","first-page":"55","DOI":"10.4161\/auto.2217","article-title":"Detection of autophagy in tissue by standard immunohistochemistry: possibilities and limitations","volume":"2","author":"Martinet","year":"2006","journal-title":"Autophagy"},{"key":"key\n\t\t\t\t20170522131404_DDM217C33","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1034\/j.1600-0854.2002.30704.x","article-title":"Induction of autophagy causes dramatic changes in the subcellular distribution of GFP-Rab24","volume":"3","author":"Munafo","year":"2002","journal-title":"Traffic"},{"key":"key\n\t\t\t\t20170522131404_DDM217C34","doi-asserted-by":"crossref","first-page":"2503","DOI":"10.1016\/j.biocel.2004.05.009","article-title":"LC3 conjugation system in mammalian autophagy","volume":"36","author":"Tanida","year":"2004","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C35","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","article-title":"Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions","volume":"95","author":"Saudou","year":"1998","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522131404_DDM217C36","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1006\/jmbi.2001.4624","article-title":"Comparison of ARM and HEAT protein repeats","volume":"309","author":"Andrade","year":"2001","journal-title":"J. Mol. Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C37","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.4161\/cc.3.12.1316","article-title":"Comparative genomics, evolution and origins of the nuclear envelope and nuclear pore complex","volume":"3","author":"Mans","year":"2004","journal-title":"Cell Cycle"},{"key":"key\n\t\t\t\t20170522131404_DDM217C38","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1016\/j.ceb.2006.06.005","article-title":"Endoplasmic reticulum stress in health and disease","volume":"18","author":"Zhao","year":"2006","journal-title":"Curr. Opin. Cell Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C39","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."},{"key":"key\n\t\t\t\t20170522131404_DDM217C40","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","author":"Omi","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"key\n\t\t\t\t20170522131404_DDM217C41","doi-asserted-by":"crossref","first-page":"36876","DOI":"10.1074\/jbc.274.52.36876","article-title":"Identification of the endoplasmic reticulum targeting signal in vesicle-associated membrane proteins","volume":"274","author":"Kim","year":"1999","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C42","doi-asserted-by":"crossref","first-page":"12062","DOI":"10.1074\/jbc.272.18.12062","article-title":"Differential interaction of nuclear receptors with the putative human transcriptional coactivator hTIF1","volume":"272","author":"Thenot","year":"1997","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C43","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/S0955-0674(00)00219-2","article-title":"Intracellular signaling from the endoplasmic reticulum to the nucleus: the unfolded protein response in yeast and mammals","volume":"13","author":"Patil","year":"2001","journal-title":"Curr. Opin. Cell Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C44","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","article-title":"A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration","volume":"23","author":"Hodgson","year":"1999","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522131404_DDM217C45","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\t20170522131404_DDM217C46","doi-asserted-by":"crossref","first-page":"19676","DOI":"10.1074\/jbc.M602802200","article-title":"Nrf1 is targeted to the endoplasmic reticulum membrane by an N-terminal transmembrane domain. Inhibition of nuclear translocation and transacting function","volume":"281","author":"Wang","year":"2006","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C47","doi-asserted-by":"crossref","first-page":"11045","DOI":"10.1073\/pnas.0604547103","article-title":"Flanking sequences profoundly alter polyglutamine toxicity in yeast","volume":"103","author":"Duennwald","year":"2006","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522131404_DDM217C48","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1523\/JNEUROSCI.1409-03.2004","article-title":"Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction","volume":"24","author":"Qin","year":"2004","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522131404_DDM217C49","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1093\/hmg\/ddi477","article-title":"Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington's disease gene results in subtle behavioral\/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitro","volume":"15","author":"Clabough","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522131404_DDM217C50","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522131404_DDM217C51","doi-asserted-by":"crossref","first-page":"2730","DOI":"10.1074\/jbc.M506751200","article-title":"Ataxin-7 Can Export from the Nucleus via a Conserved Exportin-dependent Signal","volume":"281","author":"Taylor","year":"2006","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522131404_DDM217C52","doi-asserted-by":"crossref","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":"key\n\t\t\t\t20170522131404_DDM217C53","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1083\/jcb.200503106","article-title":"Huntingtin interacting protein 1 modulates the transcriptional activity of nuclear hormone receptors","volume":"170","author":"Mills","year":"2005","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C54","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1385\/MB:32:2:147","article-title":"Mouse models of triplet repeat diseases","volume":"32","author":"Bates","year":"2006","journal-title":"Mol. Biotechnol."},{"key":"key\n\t\t\t\t20170522131404_DDM217C55","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1097\/01.wnr.0000127073.66692.8f","article-title":"Autophagosome-like vacuole formation in Huntington's disease lymphoblasts","volume":"15","author":"Nagata","year":"2004","journal-title":"Neuroreport"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/16\/21\/2600\/17244144\/ddm217.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,2]],"date-time":"2019-05-02T03:58:11Z","timestamp":1556769491000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddm217"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,8,8]]},"references-count":55,"journal-issue":{"issue":"21","published-online":{"date-parts":[[2007,8,8]]},"published-print":{"date-parts":[[2007,11,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddm217","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2007,8,8]]}}}