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Genetic Rodent Models of Huntington Disease

  • J. Stricker-Shaver
  • A. Novati
  • L. Yu-Taeger
  • H. P. Nguyen
Chapter
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1049)

Abstract

The monogenic nature of Huntington disease (HD) has led to the development of a spectrum of useful genetically modified models. In particular, rodents have pioneered as the first HD model being generated and have since been the most widely used animal model for HD in both basic research and preclinical therapeutic studies. Based on the generation strategies, these rodent models can be classified into 3 major groups, the transgenic fragment models, the transgenic full-length models and the knock-in models. These models display a range of HD-like characteristics which resemble the clinical symptoms of HD patients. Their applications in research are thus regarded as an invaluable approach to speeding up the unraveling of the underlying pathological mechanisms of HD and for finding a disease-modifying treatment for this devastating disease. In this chapter, the similarities and differences of the most commonly used rodent HD models and their relevance to human HD will be compared and discussed. This also serves to guide the selection of an appropriate rodent HD model according to the nature of investigation.

Keywords

Huntington disease Transgenic Knock-in Mouse Rat 

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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • J. Stricker-Shaver
    • 1
  • A. Novati
    • 1
  • L. Yu-Taeger
    • 1
  • H. P. Nguyen
    • 1
  1. 1.Institute of Medical Genetics and Applied GenomicsUniversity of TuebingenTuebingenGermany

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