Rodent genetic models of Huntington disease

MY Heng, PJ Detloff, RL Albin - Neurobiology of disease, 2008 - Elsevier
MY Heng, PJ Detloff, RL Albin
Neurobiology of disease, 2008Elsevier
Huntington disease (HD) is a dominantly inherited human neurodegenerative disorder
characterized by motor deficits, cognitive impairment, and psychiatric symptoms leading to
inexorable decline and death. Since the identification of the huntingtin gene and the
characteristic expanded CAG repeat/polyglutamine mutation, multiple murine genetic
models and one rat genetic model have been generated. These models fall into two general
categories: transgenic models with ectopic expression of the characteristic expanded CAG …
Huntington disease (HD) is a dominantly inherited human neurodegenerative disorder characterized by motor deficits, cognitive impairment, and psychiatric symptoms leading to inexorable decline and death. Since the identification of the huntingtin gene and the characteristic expanded CAG repeat/polyglutamine mutation, multiple murine genetic models and one rat genetic model have been generated. These models fall into two general categories: transgenic models with ectopic expression of the characteristic expanded CAG codon mutation, and knock-in models with expression of mutant huntingtin under control of endogenous regulatory elements. Rodent genetic models are valuable tools for studying mechanisms of pathogenesis in HD and for preclinical evaluation of possible therapies. In this mini-review, we provide a concise comparative summary of rodent genetic models of HD.
Elsevier