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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.
TLDR
The natural history of HD-related changes in the YAC128 mice has been defined, demonstrating the presence of huntingtin inclusions after the onset of behavior and neuropathological changes and making it an ideal mouse model for the assessment of neuroprotective and other therapeutic interventions. Expand
Cleavage at the Caspase-6 Site Is Required for Neuronal Dysfunction and Degeneration Due to Mutant Huntingtin
TLDR
Caspase-6-resistant mutant htt mice are protected against neurotoxicity induced by multiple stressors including NMDA, quinolinic acid (QA), and staurosporine and highlight the significant role of htt proteolysis and excitotoxicity in HD. Expand
Cognitive Dysfunction Precedes Neuropathology and Motor Abnormalities in the YAC128 Mouse Model of Huntington's Disease
TLDR
The clear pattern of cognitive dysfunction in YAC128 mice is similar to the symptoms and progression of cognitive deficits in human HD and provides the opportunity to examine the relationship between cognitive dysfunction, motor impairment, and neuropathology in HD and to assess whether potential therapies for HD can restore cognitive function. Expand
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
TLDR
A pathway directly linking disturbedCa2+ signaling and degeneration of MSN in the caudate nucleus in HD is described and it is suggested that Ca2+ and MPTP blockers may have a therapeutic potential for treatment of HD. Expand
Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.
TLDR
It is suggested that selective nuclear localization of mutant htt may contribute to the selective degeneration in HD and that appropriately regulated expression of full-length mutant htt in YAC128 mice results in a pattern of degeneration remarkably similar to human HD. Expand
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice
TLDR
The hypothesis that QUIN may play a role in the striatal and cortical neurodegeneration of HD is supported and important parallels and intriguing differences in the progressive neurochemical changes in these HD mouse models are demonstrated. Expand
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions.
TLDR
The shortstop mouse is protected from excitotoxicity, providing in vivo evidence suggesting that neurodegeneration in Huntington disease is mediated by excitOToxic mechanisms. Expand
Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain
TLDR
Backcrossing YAC128 mice from the FVB/N strain onto the C57BL/6 strain and the 129 strain revealed that striatal volume loss and motor dysfunction are penetrant on all three genetic backgrounds. Expand
Differential Susceptibility to Excitotoxic Stress in YAC128 Mouse Models of Huntington Disease between Initiation and Progression of Disease
TLDR
The results demonstrate that YAC128 mice display enhanced sensitivity to NMDA ex vivo and QUIN in vivo before obvious phenotypic changes in HD, and suggest that therapeutic approaches to HD may need to be altered, depending on the stage and development of the disease. Expand
Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease
TLDR
It is found that application of glutamate induced rapid loss of mitochondrial membrane potential in YAC128 MSNs, which is consistent with the hypothesis that disturbed neuronal Ca(2+) signaling plays a significant role in the degeneration of MSN containing full-length mutant Htt(exp). Expand
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