title: Designing primate models to assess the prodromal phase of Huntington's disease.
abstract: Huntington's disease (HD) is an inherited autosomal-dominant neurodegenerative disorder characterized by frontal-type cognitive deficits, involuntary choreiform movements and progressive neuronal degeneration, primarily affecting the caudate-putamen complex. There is currently no effective therapy for this disorder. Numerous efforts are directed towards the search and validation of new therapies to prevent or slow down disease progression. To this end, different animal models, including in nonhuman primates, have been developed to mimic the early phase of neuronal dysfunction that precedes degeneration in this pathology. The present manuscript provides a critical evaluation of existing and currently developed primate models of Huntington's disease. Their pertinence and predictability for the evaluation of innovative therapeutic strategies are also discussed.
authors.affiliation: Atomic energy commission (CEA), Institute of biomedical imaging (I2BM), Molecular imaging research center (MIRCen), 18 route du Panorama, 92260 Fontenay-Aux-Roses, France.
authors.forename: R
authors.initials: R
authors.lastname: Aron Badin
authors.forename: P
authors.initials: P
authors.lastname: Hantraye
mesh_terms: D000818:Animals; D058070:Asymptomatic Diseases / Q000628:therapy; D004195:Disease Models, Animal; D006801:Humans; D006816:Huntington Disease / Q000473:pathology* / Q000628:therapy; D011323:Primates; D062706:Prodromal Symptoms; D054928:Validation Studies as Topic