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• W2056531691 startingPage "13589" @default.
• W2056531691 abstract "Huntington's disease (HD) is an autosomal dominant neurodegenerative disease resulting from the expansion of a glutamine repeat in the huntingtin (Htt) protein. Current therapies are directed at managing symptoms such as chorea and psychiatric disturbances. In an effort to develop a therapy directed at disease prevention we investigated the utility of highly specific, anti-Htt intracellular antibodies (intrabodies). We previously showed that V(L)12.3, an intrabody recognizing the N terminus of Htt, and Happ1, an intrabody recognizing the proline-rich domain of Htt, both reduce mHtt-induced toxicity and aggregation in cell culture and brain slice models of HD. Due to the different mechanisms of action of these two intrabodies, we then tested both in the brains of five mouse models of HD using a chimeric adeno-associated virus 2/1 (AAV2/1) vector with a modified CMV enhancer/chicken beta-actin promoter. V(L)12.3 treatment, while beneficial in a lentiviral model of HD, has no effect on the YAC128 HD model and actually increases severity of phenotype and mortality in the R6/2 HD model. In contrast, Happ1 treatment confers significant beneficial effects in a variety of assays of motor and cognitive deficits. Happ1 also strongly ameliorates the neuropathology found in the lentiviral, R6/2, N171-82Q, YAC128, and BACHD models of HD. Moreover, Happ1 significantly prolongs the life span of N171-82Q mice. These results indicate that increasing the turnover of mHtt using AAV-Happ1 gene therapy represents a highly specific and effective treatment in diverse mouse models of HD." @default.
• W2056531691 created "2016-06-24" @default.
• W2056531691 creator A5003624509 @default.
• W2056531691 creator A5037261417 @default.
• W2056531691 creator A5071902829 @default.
• W2056531691 date "2009-10-28" @default.
• W2056531691 modified "2023-10-17" @default.
• W2056531691 title "Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease" @default.
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• W2056531691 doi "https://doi.org/10.1523/jneurosci.4286-09.2009" @default.
• W2056531691 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2822643" @default.
• W2056531691 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/19864571" @default.
• W2056531691 hasPublicationYear "2009" @default.
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