Hprt (CAG)146 mice: Age of onset of behavioral abnormalities, time course of neuronal intranuclear inclusion accumulation, neurotransmitter marker alterations, mitochondrial function markers, and susceptibility to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine
dc.contributor.author | Tallaksen-Greene, Sara J. | en_US |
dc.contributor.author | Ordway, Jared M. | en_US |
dc.contributor.author | Crouse, Andrew B. | en_US |
dc.contributor.author | Jackson, Walker S. | en_US |
dc.contributor.author | Detloff, Peter J. | en_US |
dc.contributor.author | Albin, Roger L. | en_US |
dc.date.accessioned | 2006-04-19T13:35:46Z | |
dc.date.available | 2006-04-19T13:35:46Z | |
dc.date.issued | 2003-10-13 | en_US |
dc.identifier.citation | Tallaksen-Greene, Sara J.; Ordway, Jared M.; Crouse, Andrew B.; Jackson, Walker S.; Detloff, Peter J.; Albin, Roger L. (2003)."Hprt (CAG)146 mice: Age of onset of behavioral abnormalities, time course of neuronal intranuclear inclusion accumulation, neurotransmitter marker alterations, mitochondrial function markers, and susceptibility to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine." The Journal of Comparative Neurology 465(2): 205-219. <http://hdl.handle.net/2027.42/34468> | en_US |
dc.identifier.issn | 0021-9967 | en_US |
dc.identifier.issn | 1096-9861 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/34468 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=12949782&dopt=citation | en_US |
dc.description.abstract | We reported previously a model of polyglutamine repeat disorders with insertion of 146 CAG repeats into the murine hypoxanthine phosphoribosyl transferase locus (Hprt (CAG)146 ; Ordway et al. [ 1997 ] Cell 91:753–763), which does not normally contain polyglutamine repeats. These mice develop an adult-onset neurologic phenotype of incoordination, involuntary limb clasping, seizures, and premature death. Histologic analysis demonstrates widespread ubiquinated neuronal intranuclear inclusions (NIIs). We now report characterization of the age of onset of behavioral abnormalities, correlated with the time course of occurrence of NIIs in several brain regions, and the occurrence of NIIs in non-neuronal tissues. Onset of behavioral abnormalities occurred at approximately 22 weeks of age. There was variable time course of expression of NIIs in several brain regions. Assessment of several non-neuronal tissues revealed nuclear inclusions in hepatocytes and choroid plexus epithelium. Γ-Aminobutyric acid (GABA)/benzodiazepine receptors, dopamine D1-like and D2-like receptors, and type 2 vesicular monoamine transporter (VMAT2) binding sites were assayed before and after the onset of behavioral abnormalities. GABA/benzodiazepine receptors were unchanged either before or after the onset of behavioral abnormalities in any region analyzed, whereas striatal D1-like and D2-like receptors were diminished after but not before the onset of symptoms. Dorsal striatal VMAT2 binding sites were decreased before the onset of behavioral changes. Mitochondrial electron transport chain components were assayed with histochemical methods before and after the onset of behavioral changes. There was no change in behaviorally presymptomatic or symptomatic animals. Hprt (CAG)146 mice did not exhibit increased susceptibility to the mitochondrial toxin 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Hprt (CAG)146 mice are a useful model for studying polyglutamine repeat disorders. J. Comp. Neurol. 465:205–219, 2003. © 2003 Wiley-Liss, Inc. | en_US |
dc.format.extent | 783475 bytes | |
dc.format.extent | 3118 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.language.iso | en_US | |
dc.publisher | Wiley Subscription Services, Inc., A Wiley Company | en_US |
dc.subject.other | Life and Medical Sciences | en_US |
dc.subject.other | Neuroscience, Neurology and Psychiatry | en_US |
dc.title | Hprt (CAG)146 mice: Age of onset of behavioral abnormalities, time course of neuronal intranuclear inclusion accumulation, neurotransmitter marker alterations, mitochondrial function markers, and susceptibility to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine | en_US |
dc.type | Article | en_US |
dc.rights.robots | IndexNoFollow | en_US |
dc.subject.hlbsecondlevel | Neurosciences | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109-0585 ; Drs. Tallaksen-Greene and Ordway contributed equally to this work. | en_US |
dc.contributor.affiliationum | Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109-0585 ; Geriatrics Research, Education, and Clinical Center, Ann Arbor Veterans Affairs Medical Center, Ann Arbor, Michigan 48105 ; Room 4412D, Kresge III Building, 200 Zina Pitcher Place, Ann Arbor, MI 48109-0585 | en_US |
dc.contributor.affiliationother | Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, Alabama 35294 ; Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105 | en_US |
dc.contributor.affiliationother | Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, Alabama 35294 | en_US |
dc.contributor.affiliationother | Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, Alabama 35294 | en_US |
dc.contributor.affiliationother | Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, Alabama 35294 | en_US |
dc.identifier.pmid | 12949782 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/34468/1/10855_ftp.pdf | en_US |
dc.identifier.doi | http://dx.doi.org/10.1002/cne.10855 | en_US |
dc.identifier.source | The Journal of Comparative Neurology | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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