Abstract:
:In people with Huntington's disease, an expanded CAG repeat sequence on the HTT gene confers a toxic gain function resulting in a progressive and fatal neurodegeneration. The Hdh((CAG)Q150) Huntington's disease mouse line is a knock-in model of the disease that carries ∼150 CAG repeats on the normal mouse Htt locus. To determine that these mice are a useful model of the disease, they were assessed longitudinally for motor and cognitive deficits relevant to the human disease state. Each test was conducted bi-monthly across the lifespan of the animal. The results indicate that the Hdh(Q150/Q150) mice were impaired on each of the measures used, with deficits appearing on a 3-stage water maze test at 4 months of age and on prepulse inhibition at 6 months of age, both of which were prior to the manifestation of motor abnormalities. Grip strength, as measured by the inverted cage lid test, was reduced in the Hdh(Q150/Q150) mice from 10 months of age, when the male mice also exhibited weight loss relative to their wildtype littermates. On the accelerating rotarod, deficits in the carrier mice did not appear until they were 21 months old. Our results demonstrate that the Hdh((CAG)150) is a valid model of HD that displays early and progressive cognitive deficits that precede the onset of motor abnormalities.
journal_name
Brain Res Bulljournal_title
Brain research bulletinauthors
Brooks S,Higgs G,Jones L,Dunnett SBdoi
10.1016/j.brainresbull.2010.05.004subject
Has Abstractpub_date
2012-06-01 00:00:00pages
182-8issue
2-3eissn
0361-9230issn
1873-2747pii
S0361-9230(10)00102-4journal_volume
88pub_type
杂志文章abstract::Human autopsy brain tissue is widely used to study neurodegenerative diseases such as Alzheimer's, Parkinson's and other diseases. However, when it comes to an evaluation of data obtained from such tissue, it is essential to consider potential postmortem effects on protein composition, posttranslational modification a...
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pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章,评审
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pub_type: 杂志文章
doi:10.1016/j.brainresbull.2007.10.020
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2008.09.020
更新日期:2009-03-30 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章,评审
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pub_type: 杂志文章
doi:10.1016/j.brainresbull.2008.11.005
更新日期:2009-03-16 00:00:00
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pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Brain research bulletin
pub_type: 杂志文章
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pub_type: 杂志文章
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