{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T05:27:19Z","timestamp":1784698039445,"version":"3.55.0"},"reference-count":46,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2006,1,1]],"date-time":"2006-01-01T00:00:00Z","timestamp":1136073600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The American Journal of Pathology"],"published-print":{"date-parts":[[2006,1]]},"DOI":"10.2353\/ajpath.2006.050619","type":"journal-article","created":{"date-parts":[[2007,3,16]],"date-time":"2007-03-16T19:36:39Z","timestamp":1174073799000},"page":"195-204","source":"Crossref","is-referenced-by-count":57,"title":["Abnormalities of Germ Cell Maturation and Sertoli Cell Cytoskeleton in Androgen Receptor 113 CAG Knock-In Mice Reveal Toxic Effects of the Mutant Protein"],"prefix":"10.1016","volume":"168","author":[{"given":"Zhigang","family":"Yu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nahid","family":"Dadgar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan","family":"Albertelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arno","family":"Scheller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger L.","family":"Albin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane M.","family":"Robins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew P.","family":"Lieberman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.2353\/ajpath.2006.050619_bib1","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1146\/annurev.neuro.23.1.217","article-title":"Glutamine repeats and neurodegeneration","volume":"23","author":"Zoghbi","year":"2000","journal-title":"Annu Rev Neurosci"},{"key":"10.2353\/ajpath.2006.050619_bib2","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/352077a0","article-title":"Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy","volume":"352","author":"La Spada","year":"1991","journal-title":"Nature"},{"key":"10.2353\/ajpath.2006.050619_bib3","doi-asserted-by":"crossref","first-page":"4778","DOI":"10.1523\/JNEUROSCI.0808-04.2004","article-title":"Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"24","author":"Chevalier-Larsen","year":"2004","journal-title":"J Neurosci"},{"key":"10.2353\/ajpath.2006.050619_bib4","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/S0896-6273(02)00834-6","article-title":"Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"35","author":"Katsuno","year":"2002","journal-title":"Neuron"},{"key":"10.2353\/ajpath.2006.050619_bib5","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1093\/hmg\/11.18.2103","article-title":"A mouse model of spinal and bulbar muscular atrophy","volume":"11","author":"McManamny","year":"2002","journal-title":"Hum Mol Genet"},{"key":"10.2353\/ajpath.2006.050619_bib6","doi-asserted-by":"crossref","first-page":"8389","DOI":"10.1074\/jbc.M311761200","article-title":"Androgen receptor acetylation site mutations cause trafficking defects, misfolding, and aggregation similar to expanded glutamine tracts","volume":"279","author":"Thomas","year":"2004","journal-title":"J Biol Chem"},{"key":"10.2353\/ajpath.2006.050619_bib7","doi-asserted-by":"crossref","first-page":"2197","DOI":"10.1093\/hmg\/9.14.2197","article-title":"CREB-binding protein sequestration by expanded polyglutamine","volume":"9","author":"McCampbell","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.2353\/ajpath.2006.050619_bib8","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/S0896-6273(03)00569-5","article-title":"Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport","volume":"40","author":"Szebenyi","year":"2003","journal-title":"Neuron"},{"key":"10.2353\/ajpath.2006.050619_bib9","doi-asserted-by":"crossref","first-page":"1418","DOI":"10.1096\/fj.01-1035fje","article-title":"Androgen receptor with elongated polyglutamine tract forms aggregates that alter axonal trafficking and mitochondrial distribution in motor neuronal processes","volume":"16","author":"Piccioni","year":"2002","journal-title":"FASEB J"},{"key":"10.2353\/ajpath.2006.050619_bib10","doi-asserted-by":"crossref","first-page":"21264","DOI":"10.1074\/jbc.M500144200","article-title":"The unfolded protein response modulates toxicity of the expanded glutamine androgen receptor","volume":"280","author":"Thomas","year":"2005","journal-title":"J Biol Chem"},{"key":"10.2353\/ajpath.2006.050619_bib11","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":"10.2353\/ajpath.2006.050619_bib12","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1002\/(SICI)1097-4598(200006)23:6<843::AID-MUS2>3.0.CO;2-8","article-title":"Triplet repeat expansion in neuromuscular disease","volume":"23","author":"Lieberman","year":"2000","journal-title":"Muscle Nerve"},{"key":"10.2353\/ajpath.2006.050619_bib13","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1098\/rstb.1999.0461","article-title":"Androgen receptor mutation in Kennedy's disease","volume":"354","author":"Fischbeck","year":"1999","journal-title":"Philos Trans R Soc Lond B Biol Sci"},{"key":"10.2353\/ajpath.2006.050619_bib14","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1093\/hmg\/4.4.523","article-title":"Evidence for a repressive function of the long polyglutamine tract in the human androgen receptor: possible pathogenetic relevance for the (CAG)n-expanded neuronopathies","volume":"4","author":"Kazemi-Esfarjani","year":"1995","journal-title":"Hum Mol Genet"},{"key":"10.2353\/ajpath.2006.050619_bib15","doi-asserted-by":"crossref","first-page":"1967","DOI":"10.1093\/hmg\/11.17.1967","article-title":"Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor","volume":"11","author":"Lieberman","year":"2002","journal-title":"Hum Mol Genet"},{"key":"10.2353\/ajpath.2006.050619_bib16","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1038\/ng1093-184","article-title":"Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy","volume":"5","author":"Mhatre","year":"1993","journal-title":"Nat Genet"},{"key":"10.2353\/ajpath.2006.050619_bib17","doi-asserted-by":"crossref","first-page":"3181","DOI":"10.1093\/nar\/22.15.3181","article-title":"The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function","volume":"22","author":"Chamberlain","year":"1994","journal-title":"Nucleic Acids Res"},{"key":"10.2353\/ajpath.2006.050619_bib18","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1038\/3822","article-title":"Role of Ahch in gonadal development and gametogenesis","volume":"20","author":"Yu","year":"1998","journal-title":"Nat Genet"},{"key":"10.2353\/ajpath.2006.050619_bib19","doi-asserted-by":"crossref","first-page":"2071","DOI":"10.1101\/gad.7.11.2071","article-title":"Perinatal lethality and defects in hindbrain development in mice homozygous for a targeted mutation of the zinc finger gene Krox20","volume":"7","author":"Swiatek","year":"1993","journal-title":"Genes Dev"},{"key":"10.2353\/ajpath.2006.050619_bib20","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1016\/0092-8674(93)90644-6","article-title":"Independent control of immunoglobulin switch recombination at individual switch regions evidenced through Cre-loxP-mediated gene targeting","volume":"73","author":"Gu","year":"1993","journal-title":"Cell"},{"key":"10.2353\/ajpath.2006.050619_bib21","first-page":"3893","article-title":"A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length","volume":"87","author":"Dejager","year":"2002","journal-title":"J Clin Endocrinol Metab"},{"key":"10.2353\/ajpath.2006.050619_bib22","doi-asserted-by":"crossref","first-page":"3491","DOI":"10.1242\/jcs.115.17.3491","article-title":"Failure of normal adult Leydig cell development in androgen-receptor-deficient mice","volume":"115","author":"O'Shaughnessy","year":"2002","journal-title":"J Cell Sci"},{"key":"10.2353\/ajpath.2006.050619_bib23","doi-asserted-by":"crossref","first-page":"1854","DOI":"10.1210\/en.2002-0190","article-title":"The regulator of sex-limitation gene, rsl, enforces male-specific liver gene expression by negative regulation","volume":"144","author":"Tullis","year":"2003","journal-title":"Endocrinology"},{"key":"10.2353\/ajpath.2006.050619_bib24","doi-asserted-by":"crossref","first-page":"8606","DOI":"10.1073\/pnas.88.19.8606","article-title":"A single base deletion in the Tfm androgen receptor gene creates a short-lived messenger RNA that directs internal translation initiation","volume":"88","author":"Gaspar","year":"1991","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.2353\/ajpath.2006.050619_bib25","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":"10.2353\/ajpath.2006.050619_bib26","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/S0300-9084(98)80018-7","article-title":"Nuclear basic proteins in spermiogenesis","volume":"80","author":"Wouters-Tyrou","year":"1998","journal-title":"Biochimie"},{"key":"10.2353\/ajpath.2006.050619_bib27","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1038\/386713a0","article-title":"Arrest of spermatogenesis and defective breast development in mice lacking A-myb","volume":"386","author":"Toscani","year":"1997","journal-title":"Nature"},{"key":"10.2353\/ajpath.2006.050619_bib28","doi-asserted-by":"crossref","first-page":"10133","DOI":"10.1016\/S0021-9258(17)37000-X","article-title":"An acrosomal protein, sp32, in mammalian sperm is a binding protein specific for two proacrosins and an acrosin intermediate","volume":"269","author":"Baba","year":"1994","journal-title":"J Biol Chem"},{"key":"10.2353\/ajpath.2006.050619_bib29","doi-asserted-by":"crossref","first-page":"6876","DOI":"10.1073\/pnas.0307306101","article-title":"Infertility with defective spermatogenesis and hypotestosteronemia in male mice lacking the androgen receptor in Sertoli cells","volume":"101","author":"Chang","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.2353\/ajpath.2006.050619_bib30","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1073\/pnas.0308114100","article-title":"A Sertoli cell-selective knockout of the androgen receptor causes spermatogenic arrest in meiosis","volume":"101","author":"De Gendt","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.2353\/ajpath.2006.050619_bib31","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1242\/dev.00957","article-title":"Androgen receptor function is required in Sertoli cells for the terminal differentiation of haploid spermatids","volume":"131","author":"Holdcraft","year":"2004","journal-title":"Development"},{"key":"10.2353\/ajpath.2006.050619_bib32","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":"10.2353\/ajpath.2006.050619_bib33","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1083\/jcb.200301026","article-title":"Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A","volume":"161","author":"Xia","year":"2003","journal-title":"J Cell Biol"},{"key":"10.2353\/ajpath.2006.050619_bib34","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1126\/science.1083129","article-title":"Mutations in dynein link motor neuron degeneration to defects in retrograde transport","volume":"300","author":"Hafezparast","year":"2003","journal-title":"Science"},{"key":"10.2353\/ajpath.2006.050619_bib35","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1016\/S0092-8674(01)00363-4","article-title":"Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta","volume":"105","author":"Zhao","year":"2001","journal-title":"Cell"},{"key":"10.2353\/ajpath.2006.050619_bib36","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1086\/344210","article-title":"A kinesin heavy chain (KIF5A) mutation in hereditary spastic paraplegia (SPG10)","volume":"71","author":"Reid","year":"2002","journal-title":"Am J Hum Genet"},{"key":"10.2353\/ajpath.2006.050619_bib37","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1016\/S0896-6273(02)00696-7","article-title":"Disruption of dynein\/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration","volume":"34","author":"LaMonte","year":"2002","journal-title":"Neuron"},{"key":"10.2353\/ajpath.2006.050619_bib38","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1038\/ng1123","article-title":"Mutant dynactin in motor neuron disease","volume":"33","author":"Puls","year":"2003","journal-title":"Nat Genet"},{"key":"10.2353\/ajpath.2006.050619_bib39","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":"10.2353\/ajpath.2006.050619_bib40","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":"10.2353\/ajpath.2006.050619_bib41","doi-asserted-by":"crossref","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","article-title":"Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro","volume":"24","author":"Trushina","year":"2004","journal-title":"Mol Cell Biol"},{"key":"10.2353\/ajpath.2006.050619_bib42","doi-asserted-by":"crossref","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":"10.2353\/ajpath.2006.050619_bib43","doi-asserted-by":"crossref","first-page":"1883","DOI":"10.1083\/jcb.143.7.1883","article-title":"Aggresomes: a cellular response to misfolded proteins","volume":"143","author":"Johnston","year":"1998","journal-title":"J Cell Biol"},{"key":"10.2353\/ajpath.2006.050619_bib44","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1083\/jcb.146.6.1239","article-title":"Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera","volume":"146","author":"Garcia-Mata","year":"1999","journal-title":"J Cell Biol"},{"key":"10.2353\/ajpath.2006.050619_bib45","doi-asserted-by":"crossref","first-page":"15179","DOI":"10.1073\/pnas.261400698","article-title":"Histone deacetylase inhibitors reduce polyglutamine toxicity","volume":"98","author":"McCampbell","year":"2001","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.2353\/ajpath.2006.050619_bib46","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1093\/hmg\/8.5.731","article-title":"Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone","volume":"8","author":"Stenoien","year":"1999","journal-title":"Hum Mol Genet"}],"container-title":["The American Journal of Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002944010620834?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002944010620834?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,8,11]],"date-time":"2021-08-11T08:25:08Z","timestamp":1628670308000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0002944010620834"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,1]]},"references-count":46,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2006,1]]}},"alternative-id":["S0002944010620834"],"URL":"https:\/\/doi.org\/10.2353\/ajpath.2006.050619","relation":{},"ISSN":["0002-9440"],"issn-type":[{"value":"0002-9440","type":"print"}],"subject":[],"published":{"date-parts":[[2006,1]]}}}