Neurobiology of Disease Longitudinal Evaluation of the Hdh Knock-In Murine Model of Huntington’s Disease
نویسندگان
چکیده
Mary Y. Heng,1,2 Sara J. Tallaksen-Greene,2 Peter J. Detloff,3 and Roger L. Albin1,2,4 1Neuroscience Graduate Program and 2Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, 3Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, Alabama 36294, and 4Geriatrics Research, Education, and Clinical Center, Ann Arbor Veterans Administration Medical Center, Ann Arbor, Michigan 48105
منابع مشابه
Neurobiology of Disease Brain-Derived Neurotrophic Factor Restores Synaptic Plasticity in a Knock-In Mouse Model of Huntington’s Disease
Asymptomatic Huntington’s disease (HD) patients exhibit memory and cognition deficits that generally worsen with age. Similarly, long-term potentiation (LTP), a form of synaptic plasticity involved in memory encoding, is impaired in HD mouse models well before motor disturbances occur. The reasons why LTP deteriorates are unknown. Here we show that LTP is impaired in hippocampal slices from pre...
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Asymptomatic Huntington's disease (HD) patients exhibit memory and cognition deficits that generally worsen with age. Similarly, long-term potentiation (LTP), a form of synaptic plasticity involved in memory encoding, is impaired in HD mouse models well before motor disturbances occur. The reasons why LTP deteriorates are unknown. Here we show that LTP is impaired in hippocampal slices from pre...
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Huntington’s disease (HD) is initiated by an abnormally expanded polyglutamine stretch in the huntingtin protein, conferring a novel property on the protein that leads to the loss of striatal neurons. Defects in mitochondrial function have been implicated in the pathogenesis of HD. Here, we have examined the hypothesis that the mutant huntingtin protein may directly interact with the mitochondr...
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Background & Aims: Nearly 30 hereditary disorders in humans result from an increase in the number of copies of simple repeats in genomic DNA, including fragile X syndrome, myotonic dystrophy, Huntington’s disease, and Friedreich’s ataxia. One the most frequently occurring types of mutation is trinucleotide repeat expansion. The present study was conducted with the aim of investigating the cause...
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