Annals of Neurology · 2002 · 331 citations · 31 references
Life SciencesNeurologyNeuroscienceDementiaNeurobiology Of DiseaseNeurogeneticsHuntington DiseaseMolecular Basis Of NeurodegenerationNeurodegenerative DiseasesNeurodegenerative DisordersNeurogenetic DisordersRodent GeneticsEnrichmentHuman Disease ModelsHuntington Disease DementiaHuntington's Disease ModelsNeurodegeneration ModelsRodent Environmental EnrichmentTransgenic MiceTransgenic Mouse Models
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.
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Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain
Marian DiFiglia, Ellen Sapp, Kathryn Chase et al. · Science · 1997 · 2.9K citations