{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T23:05:29Z","timestamp":1794006329452,"version":"4.8.0"},"reference-count":137,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2012,12,1]],"date-time":"2012-12-01T00:00:00Z","timestamp":1354320000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2012,12,1]],"date-time":"2012-12-01T00:00:00Z","timestamp":1354320000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100002241","name":"Japan Science and Technology Agency","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002241","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003382","name":"Core Research for Evolutional Science and Technology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003382","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003914","name":"Kennedy&apos;s Disease Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003914","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Progress in Neurobiology"],"published-print":{"date-parts":[[2012,12]]},"DOI":"10.1016\/j.pneurobio.2012.05.007","type":"journal-article","created":{"date-parts":[[2012,5,16]],"date-time":"2012-05-16T04:27:13Z","timestamp":1337142433000},"page":"246-256","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":111,"title":["Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)"],"prefix":"10.1016","volume":"99","author":[{"given":"Masahisa","family":"Katsuno","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiaki","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Adachi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruhiko","family":"Banno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirohisa","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gen","family":"Sobue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.pneurobio.2012.05.007_bib0005","doi-asserted-by":"crossref","first-page":"1039","DOI":"10.1093\/hmg\/10.10.1039","article-title":"Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death","volume":"10","author":"Adachi","year":"2001","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0010","doi-asserted-by":"crossref","first-page":"2203","DOI":"10.1523\/JNEUROSCI.23-06-02203.2003","article-title":"Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein","volume":"23","author":"Adachi","year":"2003","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0015","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1093\/brain\/awh381","article-title":"Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients","volume":"128","author":"Adachi","year":"2005","journal-title":"Brain"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0020","doi-asserted-by":"crossref","first-page":"5115","DOI":"10.1523\/JNEUROSCI.1242-07.2007","article-title":"CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model","volume":"27","author":"Adachi","year":"2007","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0025","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.1093\/hmg\/6.12.2005","article-title":"Rethinking genotype and phenotype correlations in polyglutamine expansion disorders","volume":"6","author":"Andrew","year":"1997","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0030","doi-asserted-by":"crossref","first-page":"1446","DOI":"10.1093\/brain\/awl096","article-title":"Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients","volume":"129","author":"Atsuta","year":"2006","journal-title":"Brain"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0035","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1093\/hmg\/11.5.515","article-title":"Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy","volume":"11","author":"Bailey","year":"2002","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0040","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1002\/ana.20735","article-title":"Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker","volume":"59","author":"Banno","year":"2006","journal-title":"Annals of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0045","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1002\/ana.21540","article-title":"Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy","volume":"65","author":"Banno","year":"2009","journal-title":"Annals of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0050","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1097\/00131402-200306000-00001","article-title":"Kennedy's disease initially manifesting as an endocrine disorder","volume":"4","author":"Battaglia","year":"2003","journal-title":"Journal of Clinical Neuromuscular Disease"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0055","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1111\/j.1471-4159.2009.06302.x","article-title":"The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies","volume":"110","author":"Bauer","year":"2009","journal-title":"Journal of Neurochemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0060","first-page":"301","article-title":"Lessons to be learned from the androgen receptor","volume":"11","author":"Brinkmann","year":"2001","journal-title":"European Journal of Dermatology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0065","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1038\/nrn1989","article-title":"Histone deacetylase inhibitors as therapeutics for polyglutamine disorders","volume":"7","author":"Butler","year":"2006","journal-title":"Nature Reviews Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0070","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1002\/mus.21310","article-title":"Serum creatine kinase levels in spinobulbar muscular atrophy and amyotrophic lateral sclerosis","volume":"40","author":"Chahin","year":"2009","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0075","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1615\/CritRevEukarGeneExpr.v5.i2.10","article-title":"Androgen receptor: an overview","volume":"5","author":"Chang","year":"1995","journal-title":"Critical Reviews in Eukaryotic Gene Expression"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0080","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":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0085","first-page":"255","article-title":"The androgen receptor CAG repeat and prostate cancer risk","volume":"81","author":"Clark","year":"2003","journal-title":"Methods in Molecular Medicine"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0090","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1038\/nn1750","article-title":"Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport","volume":"9","author":"Custer","year":"2006","journal-title":"Nature Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0095","doi-asserted-by":"crossref","first-page":"E393","DOI":"10.1210\/jc.2011-2439","article-title":"Shorter androgen receptor CAG repeat lengths associated with cryptorchidism risk among Hispanic white boys","volume":"97","author":"Davis-Dao","year":"2012","journal-title":"Journal of Clinical Endocrinology and Metabolism"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0100","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":"Journal of Clinical Endocrinology and Metabolism"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0105","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1002\/ana.410320517","article-title":"Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene","volume":"32","author":"Doyu","year":"1992","journal-title":"Annals of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0110","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/S1474-4422(10)70321-5","article-title":"Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trial","volume":"10","author":"Fernandez-Rhodes","year":"2011","journal-title":"The Lancet Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0115","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jns.2010.08.025","article-title":"Perspectives of Kennedy's disease","volume":"298","author":"Finsterer","year":"2010","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0120","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1023\/A:1005344403603","article-title":"Kennedy disease","volume":"20","author":"Fischbeck","year":"1997","journal-title":"Journal of Inherited Metabolic Disease"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0125","doi-asserted-by":"crossref","first-page":"1595","DOI":"10.1212\/WNL.36.12.1595","article-title":"Localization of the gene for X-linked spinal muscular atrophy","volume":"36","author":"Fischbeck","year":"1986","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0130","doi-asserted-by":"crossref","first-page":"3001","DOI":"10.1200\/JCO.2002.10.018","article-title":"Molecular biology of the androgen receptor","volume":"20","author":"Gelmann","year":"2002","journal-title":"Journal of Clinical Oncology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0135","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/0022-510X(95)00283-8","article-title":"X-linked bulbar and spinal muscular atrophy, or Kennedy disease: clinical, neurophysiological, neuropathological, neuropsychological and molecular study of a large family","volume":"135","author":"Guidetti","year":"1996","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0140","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1023\/A:1014580219761","article-title":"Epidemiological survey of X-linked bulbar and spinal muscular atrophy, or Kennedy disease, in the province of Reggio Emilia, Italy","volume":"17","author":"Guidetti","year":"2001","journal-title":"European Journal of Epidemiology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0145","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":"10.1016\/j.pneurobio.2012.05.007_bib0150","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1002\/mus.22291","article-title":"Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials","volume":"45","author":"Hama","year":"2012","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0155","doi-asserted-by":"crossref","first-page":"2063","DOI":"10.1002\/mds.22566","article-title":"Postural tremor in X-linked spinal and bulbar muscular atrophy","volume":"24","author":"Hanajima","year":"2009","journal-title":"Movement Disorders"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0160","doi-asserted-by":"crossref","first-page":"1012","DOI":"10.1136\/jnnp.45.11.1012","article-title":"X-linked recessive bulbospinal neuronopathy: a report of ten cases","volume":"45","author":"Harding","year":"1982","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0165","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1093\/hmg\/ddh144","article-title":"Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach","volume":"13","author":"Hay","year":"2004","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0170","doi-asserted-by":"crossref","first-page":"2181","DOI":"10.1210\/me.2002-0070","article-title":"The roles of androgen receptors and androgen-binding proteins in nongenomic androgen actions","volume":"16","author":"Heinlein","year":"2002","journal-title":"Molecular Endocrinology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0175","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1002\/mus.22119","article-title":"Evaluation of spinal and bulbar muscular atrophy by the clustering index method","volume":"44","author":"Higashihara","year":"2011","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0180","doi-asserted-by":"crossref","first-page":"2300","DOI":"10.1212\/WNL.42.12.2300","article-title":"Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy","volume":"42","author":"Igarashi","year":"1992","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0185","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/j.1468-1331.2008.02349.x","article-title":"Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy","volume":"16","author":"Inoue","year":"2009","journal-title":"European Journal of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0190","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1007\/s004150170069","article-title":"Clinical features and skewed X-chromosome inactivation in female carriers of X-linked recessive spinal and bulbar muscular atrophy","volume":"248","author":"Ishihara","year":"2001","journal-title":"Journal of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0195","doi-asserted-by":"crossref","first-page":"7341","DOI":"10.1074\/jbc.270.13.7341","article-title":"Identification of two transcription activation units in the N-terminal domain of the human androgen receptor","volume":"270","author":"Jenster","year":"1995","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0200","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1016\/j.bbadis.2012.02.006","article-title":"Toxic and non-toxic aggregates from the SBMA and normal forms of androgen receptor have distinct oligomeric structures","volume":"1822","author":"Jochum","year":"2012","journal-title":"Biochimica et Biophysica Acta"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0205","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.nbd.2008.12.009","article-title":"Recovery of function in a myogenic mouse model of spinal bulbar muscular atrophy","volume":"34","author":"Johansen","year":"2009","journal-title":"Neurobiology of Disease"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0210","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1136\/jnnp.55.5.394","article-title":"Central motor and sensory conduction in X-linked recessive bulbospinal neuronopathy","volume":"55","author":"Kachi","year":"1992","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0215","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1001\/archneur.56.12.1465","article-title":"Proton magnetic resonance spectroscopy in Kennedy syndrome","volume":"56","author":"Karitzky","year":"1999","journal-title":"Archives of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0220","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1136\/jnnp.2006.112532","article-title":"Widespread white matter changes in Kennedy disease: a voxel based morphometry study","volume":"78","author":"Kassubek","year":"2007","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0225","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":"10.1016\/j.pneurobio.2012.05.007_bib0230","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1038\/nm878","article-title":"Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"9","author":"Katsuno","year":"2003","journal-title":"Nature Medicine"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0235","doi-asserted-by":"crossref","first-page":"16801","DOI":"10.1073\/pnas.0506249102","article-title":"Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease","volume":"102","author":"Katsuno","year":"2005","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0240","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.expneurol.2006.01.021","article-title":"Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA)","volume":"200","author":"Katsuno","year":"2006","journal-title":"Experimental Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0245","doi-asserted-by":"crossref","first-page":"12106","DOI":"10.1523\/JNEUROSCI.3032-06.2006","article-title":"Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration","volume":"26","author":"Katsuno","year":"2006","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0250","doi-asserted-by":"crossref","first-page":"5702","DOI":"10.1523\/JNEUROSCI.0388-10.2010","article-title":"Disrupted transforming growth factor-beta signaling in spinal and bulbar muscular atrophy","volume":"30","author":"Katsuno","year":"2010","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0255","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1016\/S1474-4422(10)70182-4","article-title":"Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial","volume":"9","author":"Katsuno","year":"2010","journal-title":"The Lancet Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0260","first-page":"3","article-title":"A family of progressive bulbar palsy","volume":"16","author":"Kawahara","year":"1897","journal-title":"Aichi Medical School Journal"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0265","doi-asserted-by":"crossref","first-page":"4475","DOI":"10.1093\/hmg\/ddr380","article-title":"Impaired motoneuronal retrograde transport in two models of SBMA implicates two sites of androgen action","volume":"20","author":"Kemp","year":"2011","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0270","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1212\/WNL.18.7.671","article-title":"Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait","volume":"18","author":"Kennedy","year":"1968","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0275","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1136\/jnnp.2006.101899","article-title":"Administration of testosterone results in reversible deterioration in Kennedy's disease","volume":"79","author":"Kinirons","year":"2008","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0280","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/S0092-8674(00)81781-X","article-title":"Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice","volume":"95","author":"Klement","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0285","doi-asserted-by":"crossref","first-page":"8772","DOI":"10.1074\/jbc.275.12.8772","article-title":"Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract","volume":"275","author":"Kobayashi","year":"2000","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0290","doi-asserted-by":"crossref","first-page":"34918","DOI":"10.1074\/jbc.M302841200","article-title":"Kennedy's disease. Phosphorylation of the polyglutamine-expanded form of androgen receptor regulates its cleavage by caspase-3 and enhances cell death","volume":"278","author":"LaFevre-Bernt","year":"2003","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0295","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1038\/nrg2748","article-title":"Repeat expansion disease: progress and puzzles in disease pathogenesis","volume":"11","author":"La Spada","year":"2010","journal-title":"Nature Reviews Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0300","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":"10.1016\/j.pneurobio.2012.05.007_bib0305","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1002\/mus.21399","article-title":"Standard and modified statistical MUNE evaluations in spinal-bulbar muscular atrophy","volume":"40","author":"Lehky","year":"2009","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0310","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":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0315","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1002\/ana.410440216","article-title":"Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy","volume":"44","author":"Li","year":"1998","journal-title":"Annals of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0320","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/S0002-9440(10)65612-X","article-title":"Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy","volume":"153","author":"Li","year":"1998","journal-title":"American Journal of Pathology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0325","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1002\/mus.880180306","article-title":"Primary sensory neurons in X-linked recessive bulbospinal neuropathy: histopathology and androgen receptor gene expression","volume":"18","author":"Li","year":"1995","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0330","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1038\/sj.ejhg.5200656","article-title":"Multiple founder effects in spinal and bulbar muscular atrophy (SBMA, Kennedy disease) around the world","volume":"9","author":"Lund","year":"2001","journal-title":"European Journal of Human Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0335","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1093\/hmg\/9.9.1259","article-title":"Decreased expression of striatal signaling genes in a mouse model of Huntington's disease","volume":"9","author":"Luthi-Carter","year":"2000","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0340","doi-asserted-by":"crossref","first-page":"1776","DOI":"10.1093\/hmg\/ddr061","article-title":"Absence of disturbed axonal transport in spinal and bulbar muscular atrophy","volume":"20","author":"Malik","year":"2011","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0345","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1002\/mus.10295","article-title":"Fatigue and abnormal neuromuscular transmission in Kennedy's disease","volume":"27","author":"Meriggioli","year":"2003","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0350","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1038\/nchembio.694","article-title":"Identifying polyglutamine protein species in situ that best predict neurodegeneration","volume":"7","author":"Miller","year":"2011","journal-title":"Nature Chemical Biology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0355","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1093\/hmg\/ddh131","article-title":"Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"13","author":"Minamiyama","year":"2004","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0360","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1080\/13554790902842003","article-title":"Are cognitive and behavioural deficits a part of the clinical picture in Kennedy's disease? A case study","volume":"15","author":"Mirowska-Guzel","year":"2009","journal-title":"Neurocase"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0675","doi-asserted-by":"crossref","first-page":"e12922","DOI":"10.1371\/journal.pone.0012922","article-title":"Microarray analysis of gene expression by skeletal muscle of three mouse models of kennedy disease\/spinal bulbar muscular atrophy","volume":"5","author":"Mo","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0365","doi-asserted-by":"crossref","first-page":"18259","DOI":"10.1073\/pnas.0705501104","article-title":"Overexpression of wild-type androgen receptor in muscle recapitulates polyglutamine disease","volume":"104","author":"Monks","year":"2007","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0370","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1093\/hmg\/ddp115","article-title":"Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy","volume":"18","author":"Montie","year":"2009","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0375","doi-asserted-by":"crossref","first-page":"17425","DOI":"10.1523\/JNEUROSCI.3958-11.2011","article-title":"SIRT1 modulates aggregation and toxicity through deacetylation of the androgen receptor in cell models of SBMA","volume":"31","author":"Montie","year":"2011","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0380","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1038\/nn1717","article-title":"JNK mediates pathogenic effects of polyglutamine-expanded androgen receptor on fast axonal transport","volume":"9","author":"Morfini","year":"2006","journal-title":"Nature Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0385","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/nrn1587","article-title":"Modulation of neurodegeneration by molecular chaperones","volume":"6","author":"Muchowski","year":"2005","journal-title":"Nature Reviews Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0390","doi-asserted-by":"crossref","first-page":"21296","DOI":"10.1074\/jbc.M109.011494","article-title":"Small ubiquitin-like modifier (SUMO) modification of the androgen receptor attenuates polyglutamine-mediated aggregation","volume":"284","author":"Mukherjee","year":"2009","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0395","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1038\/nsmb1215","article-title":"A toxic monomeric conformer of the polyglutamine protein","volume":"14","author":"Nagai","year":"2007","journal-title":"Nature Structural & Molecular Biology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0400","doi-asserted-by":"crossref","first-page":"188","DOI":"10.2174\/138920110790909650","article-title":"Induction of molecular chaperones as a therapeutic strategy for the polyglutamine diseases","volume":"11","author":"Nagai","year":"2010","journal-title":"Current Pharmaceutical Biotechnology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0405","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0022-510X(88)90240-7","article-title":"Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers","volume":"87","author":"Nagashima","year":"1988","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0410","doi-asserted-by":"crossref","first-page":"936","DOI":"10.1016\/j.neuron.2010.08.034","article-title":"Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy","volume":"67","author":"Nedelsky","year":"2010","journal-title":"Neuron"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0415","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1126\/science.1056784","article-title":"Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity","volume":"291","author":"Nucifora","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0420","doi-asserted-by":"crossref","first-page":"35567","DOI":"10.1074\/jbc.M110.146845","article-title":"An interdomain interaction of the androgen receptor is required for its aggregation and toxicity in spinal and bulbar muscular atrophy","volume":"285","author":"Orr","year":"2010","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0680","doi-asserted-by":"crossref","first-page":"35567","DOI":"10.1074\/jbc.M110.146845","article-title":"An interdomain interaction of the androgen receptor is required for its aggregation and toxicity in spinal and bulbar muscular atrophy","volume":"285","author":"Orr","year":"2011","journal-title":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0425","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1146\/annurev.neuro.29.051605.113042","article-title":"Trinucleotide repeat disorders","volume":"30","author":"Orr","year":"2007","journal-title":"Annual Review of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0430","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1016\/j.clinph.2006.10.006","article-title":"Upper motor neuron involvement in X-linked recessive bulbospinal muscular atrophy","volume":"118","author":"Pachatz","year":"2007","journal-title":"Clinical Neurophysiology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0435","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1093\/hmg\/ddm109","article-title":"Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity","volume":"16","author":"Palazzolo","year":"2007","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0440","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.jsbmb.2007.09.016","article-title":"The role of the polyglutamine tract in androgen receptor","volume":"108","author":"Palazzolo","year":"2008","journal-title":"Journal of Steroid Biochemistry and Molecular Biology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0445","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.neuron.2009.07.019","article-title":"Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy","volume":"63","author":"Palazzolo","year":"2009","journal-title":"Neuron"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0450","doi-asserted-by":"crossref","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":"10.1016\/j.pneurobio.2012.05.007_bib0455","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1212\/WNL.49.2.568","article-title":"Spinobulbar muscular atrophy can mimic ALS: the importance of genetic testing in male patients with atypical ALS","volume":"49","author":"Parboosingh","year":"1997","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0460","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.yfrne.2011.06.003","article-title":"Neurotoxic effects of androgens in spinal and bulbar muscular atrophy","volume":"32","author":"Parodi","year":"2011","journal-title":"Frontiers in Neuroendocrinology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0465","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1006\/mcne.1999.0773","article-title":"Nuclear targeting of mutant Huntingtin increases toxicity","volume":"14","author":"Peters","year":"1999","journal-title":"Molecular and Cellular Neurosciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0470","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 Journal"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0475","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.yfrne.2004.03.001","article-title":"The polyglutamine tract of androgen receptor: from functions to dysfunctions in motor neurons","volume":"25","author":"Poletti","year":"2004","journal-title":"Frontiers in Neuroendocrinology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0480","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":"Nature Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0485","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1093\/hmg\/ddn310","article-title":"Mitochondrial abnormalities in spinal and bulbar muscular atrophy","volume":"18","author":"Ranganathan","year":"2009","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0490","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1016\/j.tips.2010.08.005","article-title":"Therapeutic approaches to spinal and bulbar muscular atrophy","volume":"31","author":"Ranganathan","year":"2010","journal-title":"Trends in Pharmacological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0495","doi-asserted-by":"crossref","first-page":"3242","DOI":"10.1093\/brain\/awp258","article-title":"Clinical features of spinal and bulbar muscular atrophy","volume":"132","author":"Rhodes","year":"2009","journal-title":"Brain"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0500","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1002\/mus.22159","article-title":"Subclinical autonomic dysfunction in spinobulbar muscular atrophy (Kennedy disease)","volume":"44","author":"Rocchi","year":"2011","journal-title":"Muscle and Nerve"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0505","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/S0896-6273(02)00872-3","article-title":"Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders","volume":"35","author":"Ross","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0510","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.nbd.2010.08.023","article-title":"17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy","volume":"41","author":"Rusmini","year":"2011","journal-title":"Neurobiology of Disease"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0515","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":"10.1016\/j.pneurobio.2012.05.007_bib0520","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1212\/WNL.59.5.770","article-title":"Expression of X-linked bulbospinal muscular atrophy (Kennedy disease) in two homozygous women","volume":"59","author":"Schmidt","year":"2002","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0525","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1093\/hmg\/9.1.133","article-title":"Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract","volume":"9","author":"Simeoni","year":"2000","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0530","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1111\/j.1365-2133.2007.08026.x","article-title":"Men with Kennedy disease have a reduced risk of androgenetic alopecia","volume":"157","author":"Sinclair","year":"2007","journal-title":"British Journal of Dermatology"},{"issue":"Pt 1","key":"10.1016\/j.pneurobio.2012.05.007_bib0535","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1093\/brain\/112.1.209","article-title":"X-linked recessive bulbospinal neuronopathy. A clinicopathological study","volume":"112","author":"Sobue","year":"1989","journal-title":"Brain"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0540","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1007\/BF00308716","article-title":"Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells","volume":"79","author":"Sobue","year":"1990","journal-title":"Acta Neuropathologica"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0545","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/0022-510X(93)90157-T","article-title":"Subclinical phenotypic expressions in heterozygous females of X-linked recessive bulbospinal neuronopathy","volume":"117","author":"Sobue","year":"1993","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0550","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0896-6273(04)00082-0","article-title":"Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration","volume":"41","author":"Sopher","year":"2004","journal-title":"Neuron"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0555","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1016\/j.jns.2007.08.012","article-title":"Spinal and bulbar muscular atrophy: skeletal muscle pathology in male patients and heterozygous females","volume":"264","author":"Soraru","year":"2008","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0560","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1001\/archneur.59.12.1921","article-title":"X-linked bulbospinal neuronopathy: Kennedy disease","volume":"59","author":"Sperfeld","year":"2002","journal-title":"Archives of Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0565","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1212\/01.WNL.0000151978.74467.E7","article-title":"Laryngospasm: an underdiagnosed symptom of X-linked spinobulbar muscular atrophy","volume":"64","author":"Sperfeld","year":"2005","journal-title":"Neurology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0570","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1038\/35099568","article-title":"Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila","volume":"413","author":"Steffan","year":"2001","journal-title":"Nature"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0575","doi-asserted-by":"crossref","first-page":"3818","DOI":"10.1073\/pnas.0809819106","article-title":"Aberrant E2F activation by polyglutamine expansion of androgen receptor in SBMA neurotoxicity","volume":"106","author":"Suzuki","year":"2009","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0580","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1093\/brain\/awm289","article-title":"CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA","volume":"131","author":"Suzuki","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0585","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1136\/jnnp.2009.190462","article-title":"The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy","volume":"81","author":"Suzuki","year":"2010","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0590","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":"10.1016\/j.pneurobio.2012.05.007_bib0595","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1016\/S0896-6273(02)00875-9","article-title":"Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila","volume":"35","author":"Takeyama","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0600","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1093\/hmg\/5.9.1253","article-title":"Founder effect in spinal and bulbar muscular atrophy (SBMA)","volume":"5","author":"Tanaka","year":"1996","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0605","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1086\/302578","article-title":"Tissue-specific somatic mosaicism in spinal and bulbar muscular atrophy is dependent on CAG-repeat length and androgen receptor--gene expression level","volume":"65","author":"Tanaka","year":"1999","journal-title":"American Journal of Human Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0610","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1093\/hmg\/ddg074","article-title":"Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein","volume":"12","author":"Taylor","year":"2003","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0615","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":"Journal of Biological Chemistry"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0620","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1093\/hmg\/ddn419","article-title":"17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse","volume":"18","author":"Tokui","year":"2009","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0625","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1111\/j.1600-0404.1998.tb01732.x","article-title":"High prevalence of Kennedy's disease in Western Finland \u2013 is the syndrome underdiagnosed?","volume":"98","author":"Udd","year":"1998","journal-title":"Acta Neurologica Scandinavica"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0630","doi-asserted-by":"crossref","first-page":"1727","DOI":"10.1002\/hbm.20971","article-title":"Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging","volume":"31","author":"Unrath","year":"2010","journal-title":"Human Brain Mapping"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0635","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1210\/en.2009-1036","article-title":"Androgen regulates brain-derived neurotrophic factor in spinal motoneurons and their target musculature","volume":"151","author":"Verhovshek","year":"2010","journal-title":"Endocrinology"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0640","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1038\/nm1298","article-title":"17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration","volume":"11","author":"Waza","year":"2005","journal-title":"Nature Medicine"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0645","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1385\/JMN:23:1-2:069","article-title":"Role of heat shock proteins during polyglutamine neurodegeneration: mechanisms and hypothesis","volume":"23","author":"Wyttenbach","year":"2004","journal-title":"Journal of Molecular Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0650","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/S0022-510X(96)00303-6","article-title":"X-linked spinal and bulbar muscular atrophy with myasthenic symptoms","volume":"146","author":"Yamada","year":"1997","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0655","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1038\/nm1547","article-title":"ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor","volume":"13","author":"Yang","year":"2007","journal-title":"Nature Medicine"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0660","doi-asserted-by":"crossref","first-page":"1987","DOI":"10.1523\/JNEUROSCI.4072-08.2009","article-title":"Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5\/Hrk","volume":"29","author":"Young","year":"2009","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0665","doi-asserted-by":"crossref","first-page":"2663","DOI":"10.1172\/JCI28773.","article-title":"Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model","volume":"116","author":"Yu","year":"2006","journal-title":"Journal of Clinical Investigation"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0685","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1242\/dmm.003301","article-title":"Altered RNA splicing contributes to skeletal muscle pathology in Kennedy disease knock-in mice","volume":"2","author":"Yu","year":"2009","journal-title":"Disease Models & Mechanisms"},{"key":"10.1016\/j.pneurobio.2012.05.007_bib0670","doi-asserted-by":"crossref","first-page":"e1002321","DOI":"10.1371\/journal.pgen.1002321","article-title":"Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice","volume":"7","author":"Yu","year":"2011","journal-title":"PLoS Genetics"}],"container-title":["Progress in Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0301008212000743?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0301008212000743?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,4]],"date-time":"2025-10-04T03:55:24Z","timestamp":1759550124000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0301008212000743"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,12]]},"references-count":137,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2012,12]]}},"alternative-id":["S0301008212000743"],"URL":"https:\/\/doi.org\/10.1016\/j.pneurobio.2012.05.007","relation":{},"ISSN":["0301-0082"],"issn-type":[{"value":"0301-0082","type":"print"}],"subject":[],"published":{"date-parts":[[2012,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)","name":"articletitle","label":"Article Title"},{"value":"Progress in Neurobiology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.pneurobio.2012.05.007","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2012 Elsevier Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}