title: Environmental enrichment slows disease progression in R6/2 Huntington's disease mice.
abstract: Huntington's disease is a genetic disorder that causes motor dysfunction, personality changes, dementia, and premature death. There is currently no effective therapy. Several transgenic models of Huntington's disease are available, the most widely used of which is the R6/2 mouse, because of its rapid disease progression. Environmental enrichment alters gene expression in the normal mouse brain, and modulates the course of several neurological disorders. Environmentally enriched mice may actually mimic human disease more accurately. We found that even limited environmental enrichment slows decline in RotaRod performance in R6/2 mice, despite rapid disease progression, whereas in normal littermates, maximal enrichment was required to induce a marked improvement in behavioral tests. Enrichment also delayed the loss of peristriatal cerebral volume in R6/2 brains. These results could provide the basis for a rational approach to ameliorate the effects of Huntington's disease.
authors.affiliation: Division of Medical and Molecular Genetics, GKT School of Medicine, King's College London, UK. ehockly@hotmail.com
authors.forename: Emma
authors.initials: E
authors.lastname: Hockly
authors.forename: Patricia M
authors.initials: PM
authors.lastname: Cordery
authors.forename: Benjamin
authors.initials: B
authors.lastname: Woodman
authors.forename: Amarbirpal
authors.initials: A
authors.lastname: Mahal
authors.forename: Anton
authors.initials: A
authors.lastname: van Dellen
authors.forename: Colin
authors.initials: C
authors.lastname: Blakemore
authors.forename: Cathryn M
authors.initials: CM
authors.lastname: Lewis
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Hannan
authors.forename: Gillian P
authors.initials: GP
authors.lastname: Bates
mesh_terms: D000818:Animals; D001522:Behavior, Animal; D002540:Cerebral Cortex / Q000473:pathology; D003342:Corpus Striatum / Q000473:pathology; D003594:Cytoplasmic Granules / Q000737:chemistry; D018450:Disease Progression; D004779:Environment Design; D005260:Female; D018737:Hand Strength; D006816:Huntington Disease / Q000235:genetics / Q000473:pathology* / Q000628:therapy*; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008808:Mice, Inbred CBA; D008822:Mice, Transgenic; D009043:Motor Activity; D010455:Peptides / Q000235:genetics; D010641:Phenotype; D011110:Polymorphism, Genetic; D025801:Ubiquitin / Q000032:analysis; D015431:Weight Loss
chemical_list: D010455:Peptides; D025801:Ubiquitin; C097188:polyglutamine