Murine models of Huntington disease

Abstract

Huntington disease is an inherited neurodegenerative disorder associated with inexorable progression. To date, no therapy has proved effective in modifying the disease course or improving survival. We present here an evaluation of our most valuable asset in the development of a cure – the mouse model. In particular, we will reflect on the relative strengths and weaknesses of current models in reprising Huntington disease pathology, evaluate their role in the development of novel therapeutic agents through preclinical trials and consider their future impact on Huntington disease research.