title: Of rodents and men: understanding the emergence of motor and cognitive symptoms in Huntington disease.
abstract: Arguably, one of the most important milestones in Huntington disease research since the discovery of the gene responsible has been the generation of different genetic animal models. Although clinical reports have shown evidence of progressive cognitive impairments in gene carriers before motor symptoms are diagnosed, such symptoms have been much less obvious in animal models. In this review, we summarize the three main classes of animal models for Huntington disease and describe some relevant translational assays for behavioural deficits evaluation. Finally, we argue that a good knowledge of the emergence of motor and cognitive symptoms in mice and rat models is indispensable for the selection of endpoint measures in early preclinical drug screening studies.
authors.affiliation: aBrain and Spine Institute (ICM), Team 'Alzheimer's and Prion Diseases', CNRS-UMR 7225 Pitié-Salpêtrière University Hospital, Paris, France bSchool of Psychology, Victoria University of Wellington, Wellington, New Zealand.
authors.forename: Yah-Se K
authors.initials: YS
authors.lastname: Abada
authors.forename: Bart A
authors.initials: BA
authors.lastname: Ellenbroek
mesh_terms: D000818:Animals; D003072:Cognition Disorders / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D004195:Disease Models, Animal; D004353:Drug Evaluation, Preclinical / Q000379:methods; D023381:Endpoint Determination; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000503:physiopathology*; D051379:Mice; D051381:Rats; D013045:Species Specificity; D057170:Translational Research, Biomedical / Q000379:methods