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Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib77","doi-asserted-by":"crossref","first-page":"649","DOI":"10.2337\/diabetes.48.3.649","article-title":"Mice transgenic for an expanded CAG repeat in the Huntington's disese gene develop diabetes meelitus","volume":"48","author":"Hurlbert","year":"1999","journal-title":"Diabetes"},{"key":"10.1016\/j.pneurobio.2007.03.004_bib78","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1046\/j.1471-4159.2000.0750830.x","article-title":"Early alterations in gene expression and cell morphology in a mouse model of Huntington's disease","volume":"75","author":"Iannicola","year":"2000","journal-title":"J. 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Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib99","doi-asserted-by":"crossref","first-page":"1911","DOI":"10.1093\/hmg\/11.17.1911","article-title":"Dysregulation of gene expression in the R6\/2 model of polyglutamine disease: parallel changes in muscle and brain","volume":"11","author":"Luthi-Carter","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib100","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1093\/hmg\/11.17.1927","article-title":"Polyglutamine and transcription: gene expression changes chared by DRPLA and Huntington's disease mouse models reveal context-independent effects","volume":"11","author":"Luthi-Carter","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib101","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1016\/j.nbd.2003.08.024","article-title":"Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA expression levels, protein abundance, interacting proteins and phosphorylation","volume":"14","author":"Luthi-Carter","year":"2003","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib102","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"},{"issue":"Suppl. A106","key":"10.1016\/j.pneurobio.2007.03.004_bib103","article-title":"Co-localization of CBP with expanded polyglutamine containing androgen receptor","volume":"65","author":"McCampbell","year":"1999","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib104","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":"10.1016\/j.pneurobio.2007.03.004_bib105","doi-asserted-by":"crossref","first-page":"4909","DOI":"10.1111\/j.1432-1033.2004.04460.x","article-title":"Structure, expression and regulation of the cannabinoid receptor gene (CB1) in Huntington's disease transgenic mice","volume":"271","author":"McCaw","year":"2004","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib106","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1038\/263517a0","article-title":"Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic acid and kainic acids","volume":"263","author":"McGeer","year":"1976","journal-title":"Nature"},{"key":"10.1016\/j.pneurobio.2007.03.004_bib107","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1016\/S0165-6147(00)01884-8","article-title":"Mouse models of Huntington's disease","volume":"23","author":"Menalled","year":"2002","journal-title":"Trends Pharmacol. Sci."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib108","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1006\/exnr.1999.7327","article-title":"Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease","volume":"162","author":"Menalled","year":"2000","journal-title":"Exp. 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Neurol."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib125","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1111\/j.1471-4159.2005.03047.x","article-title":"Brain-derived neurotrophic factor modulates dopaminergic deficits in a transgenic mouse model of Huntington's disease","volume":"93","author":"Pineda","year":"2005","journal-title":"J. Neurochem."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib126","doi-asserted-by":"crossref","first-page":"16672","DOI":"10.1074\/jbc.M511648200","article-title":"Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective","volume":"281","author":"Qiu","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib127","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1038\/2510","article-title":"Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA","volume":"20","author":"Reddy","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib128","doi-asserted-by":"crossref","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","article-title":"Differential loss of striatal projection neurons in Huntington disease","volume":"85","author":"Reiner","year":"1988","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.pneurobio.2007.03.004_bib129","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1046\/j.1471-4159.1999.721773.x","article-title":"Brain neurotransmitter deficits in mice transgenic for the Huntington's disease mutation","volume":"72","author":"Reynolds","year":"1999","journal-title":"J. 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