Longitudinal DTI reveals presymptomatic white matter changes in YAC128 mouse model of Huntington disease
نویسندگان
چکیده
Xin Hong, Ling Guo, Roy Teo, Yi Lin Tay, Xuan Vinh To, Reshmi Rajendran, Si Kang Lew, Yee Ling Tan, Yihui Huang, Michael R. Hayden, Mahmoud A. Pouladi, and Kai-Hsiang Chuang Singapore Bioimaging Consortium, Agency for Science, Technology and Research, Singapore, Singapore, Singapore, Translational Laboratory in Genetic Medicine, Agency for Science, Technology and Research, Singapore, Singapore, Department of Medicine, National University of Singapore, Singapore, Singapore
منابع مشابه
Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease.
Models of Huntington disease (HD) recapitulate some neuropathological features of the disease. However, a global natural history of neuroanatomy in a mouse expressing full-length huntingtin has not been conducted. We investigated neuropathological changes in the YAC128 murine model of HD using magnetic resonance imaging (MRI). Structures affected in human HD are reduced in the YAC128 mice both ...
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Increasing evidence supports a role for abnormal immune activation and inflammatory responses in Huntington disease (HD). In this study, we evaluated the therapeutic potential of laquinimod (1 and 10 mg/kg), a novel immunomodulatory agent shown to be protective in a number of neuroinflammatory conditions, in the YAC128 mouse model of HD. Treatment with laquinimod for 6 months rescued atrophy in...
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Huntington disease (HD) is an inherited, fatal neurodegenerative disease with no disease-modifying therapy currently available. In addition to characteristic motor deficits and atrophy of the caudate nucleus, signature hallmarks of HD include behavioral abnormalities, immune activation, and cortical and white matter loss. The identification and validation of novel therapeutic targets that contr...
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Objective To evaluate the role of the involvement of white matter tracts in huntingtin gene mutation patients as a potential biomarker of the progression of the disease. Methods We evaluated 34 participants (11 symptomatic huntingtin gene mutation, 12 presymptomatic huntingtin gene mutation, and 11 controls). We performed brain magnetic resonance imaging to assess white matter integrity using...
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References: [1] E.J. Slow, et al, “Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.,” Hum Mol Genet, vol. 12, 2003, p. 1555―67. [2] J.M. Van Raamsdonk, et al, “Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.,” Hum Mol Genet, vol. 14, 2005, p. 3823―35. [3] J.P. Lerch, et al, “Automated deformatio...
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