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Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease pp 149–160Cite as

Large Animal Models of Huntington’s Disease

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Part of the book series: Current Topics in Behavioral Neurosciences ((CTBN,volume 22))

Abstract

Huntington’s disease is caused by the expansion of a polyglutamine repeat (> 37 glutamines) in the disease protein huntingtin, which results in preferential neuronal loss in distinct brain regions. Mutant huntingtin causes late-onset neurological symptoms in patients in middle life, though the expression of mutant huntingtin is ubiquitous from early life. Thus, it is important to understand why mutant huntingtin selectively causes neuronal loss in an age-dependent manner. Transgenic animal models have been essential tools for uncovering the pathogenesis and therapeutic targets of neurodegenerative diseases. Genetic mouse models have been investigated extensively and have revealed the common pathological hallmark of age-dependent formation of aggregates or inclusions consisting of misfolded proteins. However, most genetic mouse models lack striking neurodegeneration that has been found in patient brains. Since there are considerable species differences between small and large animals, large animal models of Huntington’s disease may allow one to identify the pathological features that are more similar to those in patients and also help uncover more effective therapeutic targets. This chapter will focus on the important findings from large animal models of Huntington’s disease and discusses the use of large animal models to investigate the pathogenesis of Huntington’s disease and develop new therapeutic strategies.

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Acknowledgments

This work was supported by NIH grants NS036232, AG019206, NS041669 for X.J.L., and AG031153 for S.H.L.

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Authors and Affiliations

  1. Department of Human Genetics, Emory University School of Medicine, 615 Michael St 347, Atlanta, GA, 30322, USA

    Xiao-Jiang Li & Shihua Li

  2. Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, No.1 West Beichen Road, Chaoyang District, 100101, Beijing, China

    Xiao-Jiang Li

Authors
  1. Xiao-Jiang Li
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  2. Shihua Li
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Correspondence to Xiao-Jiang Li .

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Editors and Affiliations

  1. Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tübingen, Germany

    Hoa Huu Phuc Nguyen

  2. Dept of Experimental Medical Science, Lund University, Lund, Sweden

    M. Angela Cenci

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© 2013 Springer-Verlag Berlin Heidelberg

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Li, XJ., Li, S. (2013). Large Animal Models of Huntington’s Disease. In: Nguyen, H., Cenci, M. (eds) Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease. Current Topics in Behavioral Neurosciences, vol 22. Springer, Berlin, Heidelberg. https://doi.org/10.1007/7854_2013_246

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