{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T19:25:20Z","timestamp":1791141920043,"version":"4.1.0"},"reference-count":44,"publisher":"Oxford University Press (OUP)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,1,1]]},"DOI":"10.1093\/hmg\/ddp467","type":"journal-article","created":{"date-parts":[[2009,10,14]],"date-time":"2009-10-14T03:29:30Z","timestamp":1255490970000},"page":"65-78","source":"Crossref","is-referenced-by-count":146,"title":["Identical oligomeric and fibrillar structures captured from the brains of R6\/2 and knock-in mouse models of Huntington's disease"],"prefix":"10.1093","volume":"19","author":[{"given":"Kirupa","family":"Sathasivam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amin","family":"Lane","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin","family":"Legleiter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Warley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben","family":"Woodman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steve","family":"Finkbeiner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Paganetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J.","family":"Muchowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart","family":"Wilson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gillian P.","family":"Bates","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2009,10,12]]},"reference":[{"key":"key\n\t\t\t\t20170522165610_DDP467C1","volume-title":"Huntington's Disease","author":"Bates","year":"2002","edition":"3rd edn."},{"key":"key\n\t\t\t\t20170522165610_DDP467C2","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":"Huntington's Disease Collaborative Research Group","year":"1993","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522165610_DDP467C3","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\t20170522165610_DDP467C4","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1523\/JNEUROSCI.19-07-02522.1999","article-title":"Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology","volume":"19","author":"Gutekunst","year":"1999","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522165610_DDP467C5","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1038\/nsmb860","article-title":"Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer","volume":"11","author":"Wacker","year":"2004","journal-title":"Nat. Struct. Mol. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C6","doi-asserted-by":"crossref","first-page":"41032","DOI":"10.1074\/jbc.M205809200","article-title":"Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization","volume":"277","author":"Poirier","year":"2002","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522165610_DDP467C7","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1038\/nsmb.1570","article-title":"Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism","volume":"16","author":"Thakur","year":"2009","journal-title":"Nat. Struct. Mol. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C8","doi-asserted-by":"crossref","first-page":"2811","DOI":"10.1093\/hmg\/9.19.2811","article-title":"Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila","volume":"9","author":"Chan","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C9","doi-asserted-by":"crossref","first-page":"7841","DOI":"10.1073\/pnas.140202897","article-title":"Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils","volume":"97","author":"Muchowski","year":"2000","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522165610_DDP467C10","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1093\/hmg\/ddl210","article-title":"Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models","volume":"15","author":"Ehrnhoefer","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C11","doi-asserted-by":"crossref","first-page":"4246","DOI":"10.1073\/pnas.0511256103","article-title":"Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases","volume":"103","author":"Bodner","year":"2006","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522165610_DDP467C12","doi-asserted-by":"crossref","first-page":"9679","DOI":"10.1073\/pnas.0812083106","article-title":"Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity","volume":"106","author":"Nekooki-Machida","year":"2009","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522165610_DDP467C13","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\t20170522165610_DDP467C14","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1097\/01.wco.0000084223.82329.bb","article-title":"Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?","volume":"16","author":"Bates","year":"2003","journal-title":"Curr. Opin. Neurol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C15","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1093\/hmg\/8.7.1227","article-title":"Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice","volume":"8","author":"Li","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C16","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1006\/nbdi.2001.0438","article-title":"Inhibition of polyglutamine aggregation in R6\/2 HD brain slices-complex dose-response profiles","volume":"8","author":"Smith","year":"2001","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522165610_DDP467C17","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington's disease","volume":"10","author":"Lin","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C18","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.brainresbull.2006.11.004","article-title":"The Hdh(Q150\/Q150) knock-in mouse model of HD and the R6\/2 exon 1 model develop comparable and widespread molecular phenotypes","volume":"72","author":"Woodman","year":"2007","journal-title":"Brain Res. Bull."},{"key":"key\n\t\t\t\t20170522165610_DDP467C19","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1093\/hmg\/ddm133","article-title":"Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage","volume":"16","author":"Kuhn","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C20","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0076-6879(99)09045-X","article-title":"Detection of polyglutamine aggregation in mouse models","volume":"309","author":"Davies","year":"1999","journal-title":"Methods Enzymol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C21","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1373\/49.10.1774","article-title":"Polymeric ligands with specificity for aggregated prion proteins","volume":"49","author":"Lane","year":"2003","journal-title":"Clin. Chem."},{"key":"key\n\t\t\t\t20170522165610_DDP467C22","article-title":"Binding of pathological forms of prion proteins","author":"Lane","year":"2003"},{"key":"key\n\t\t\t\t20170522165610_DDP467C23","doi-asserted-by":"crossref","first-page":"2425","DOI":"10.1093\/hmg\/10.21.2425","article-title":"Centrosome disorganization in fibroblast cultures derived from R6\/2 Huntington's disease (HD) transgenic mice and HD patients","volume":"10","author":"Sathasivam","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C24","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1093\/hmg\/8.5.813","article-title":"Formation of polyglutamine inclusions in non-CNS tissue","volume":"8","author":"Sathasivam","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C25","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/S0361-9230(03)00185-0","article-title":"Standardization and statistical approaches to therapeutic trials in the R6\/2 mouse","volume":"61","author":"Hockly","year":"2003","journal-title":"Brain Res. Bull."},{"key":"key\n\t\t\t\t20170522165610_DDP467C26","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/S0361-9230(01)00599-8","article-title":"New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins","volume":"56","author":"Ko","year":"2001","journal-title":"Brain Res. Bull."},{"key":"key\n\t\t\t\t20170522165610_DDP467C27","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1107\/S1744309105036547","article-title":"Crystallization and diffraction properties of the Fab fragment of 3B5H10, an antibody specific for disease-causing polyglutamine stretches","volume":"61","author":"Peters-Libeu","year":"2005","journal-title":"Acta. Crystallogr. Sect. F Struct. Biol. Cryst. Commun."},{"key":"key\n\t\t\t\t20170522165610_DDP467C28","doi-asserted-by":"crossref","first-page":"2905","DOI":"10.1093\/hmg\/11.23.2905","article-title":"Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells","volume":"11","author":"Yang","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522165610_DDP467C29","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1006\/jmbi.2001.4850","article-title":"Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity","volume":"311","author":"Chen","year":"2001","journal-title":"J. Mol. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C30","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1038\/nsmb.1437","article-title":"EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers","volume":"15","author":"Ehrnhoefer","year":"2008","journal-title":"Nat. Struct. Mol. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C31","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.nano.2004.11.004","article-title":"Atomic force microscopy analysis of the Huntington protein nanofibril formation","volume":"1","author":"Dahlgren","year":"2005","journal-title":"Nanomedicine"},{"key":"key\n\t\t\t\t20170522165610_DDP467C32","doi-asserted-by":"crossref","first-page":"9361","DOI":"10.1523\/JNEUROSCI.2365-04.2004","article-title":"Biochemical, ultrastructural, and reversibility studies on huntingtin filaments isolated from mouse and human brain","volume":"24","author":"Diaz-Hernandez","year":"2004","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522165610_DDP467C33","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/S0092-8674(00)80514-0","article-title":"Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo","volume":"90","author":"Scherzinger","year":"1997","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522165610_DDP467C34","doi-asserted-by":"crossref","first-page":"4604","DOI":"10.1073\/pnas.96.8.4604","article-title":"Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology","volume":"96","author":"Scherzinger","year":"1999","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522165610_DDP467C35","doi-asserted-by":"crossref","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":"key\n\t\t\t\t20170522165610_DDP467C36","doi-asserted-by":"crossref","first-page":"3157","DOI":"10.1074\/jbc.M609972200","article-title":"Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy","volume":"282","author":"Li","year":"2007","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522165610_DDP467C37","doi-asserted-by":"crossref","first-page":"12749","DOI":"10.1021\/bi030029q","article-title":"Self-assembly of Abeta(1\u201342) into globular neurotoxins","volume":"42","author":"Chromy","year":"2003","journal-title":"Biochemistry"},{"key":"key\n\t\t\t\t20170522165610_DDP467C38","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.sbi.2007.01.007","article-title":"Polymorphism in the intermediates and products of amyloid assembly","volume":"17","author":"Kodali","year":"2007","journal-title":"Curr. Opin. Struct. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C39","doi-asserted-by":"crossref","first-page":"1344","DOI":"10.1096\/fj.04-3137fje","article-title":"Fine structure study of Abeta1\u201342 fibrillogenesis with atomic force microscopy","volume":"19","author":"Arimon","year":"2005","journal-title":"FASEB J."},{"key":"key\n\t\t\t\t20170522165610_DDP467C40","doi-asserted-by":"crossref","first-page":"11634","DOI":"10.1073\/pnas.182393899","article-title":"Inaugural article: a linear lattice model for polyglutamine in CAG-expansion diseases","volume":"99","author":"Bennett","year":"2002","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522165610_DDP467C41","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1038\/nsmb1234","article-title":"The structure of a polyQ-anti-polyQ complex reveals binding according to a linear lattice model","volume":"14","author":"Li","year":"2007","journal-title":"Nat. Struct. Mol. Biol."},{"key":"key\n\t\t\t\t20170522165610_DDP467C42","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1111\/j.1471-4159.2007.05032.x","article-title":"Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease","volume":"104","author":"Weiss","year":"2008","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522165610_DDP467C43","doi-asserted-by":"crossref","first-page":"21647","DOI":"10.1074\/jbc.M109.016923","article-title":"Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment","volume":"284","author":"Legleiter","year":"2009","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522165610_DDP467C44","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/j.jcis.2004.05.009","article-title":"In situ AFM studies of astrocyte-secreted apolipoprotein E and J-containing lipoproteins","volume":"278","author":"Legleiter","year":"2004","journal-title":"J. Colloid. Inter. Sci."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/19\/1\/65\/17250709\/ddp467.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,17]],"date-time":"2024-03-17T03:26:48Z","timestamp":1710646008000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddp467"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,10,12]]},"references-count":44,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2009,10,12]]},"published-print":{"date-parts":[[2010,1,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddp467","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2009,10,12]]}}}