Proceedings of the National Academy of Sciences · 2009 · 262 citations · 32 references
Life SciencesNeurobiologyNeuroscienceMolecular NeuroscienceNeuroplasticitySynaptic PlasticityHuntington DiseaseLong-term PotentiationNeurodegenerative DiseasesNeurodegenerative DisordersSynaptic Strength RegulationSynaptic TransmissionAmpa ReceptorsDendritic SpinesKnockout Mouse ModelsLong-term Synaptic PlasticityNeurotrophic Factor SignalingNeurotrophin-mediated PlasticityRodent Synaptic PlasticityTransgenic Mouse Models
Cognitive problems occur in asymptomatic gene carriers of Huntington's disease (HD), and mouse models of the disease exhibit impaired learning and substantial deficits in the cytoskeletal changes that stabilize long-term potentiation (LTP). The latter effects may be related to the decreased production of brain-derived neurotrophic factor (BDNF) associated with the HD mutation. This study asked whether up-regulating endogenous BDNF levels with an ampakine, a positive modulator of AMPA-type glutamate receptors, rescues plasticity and reduces learning problems in HD (CAG140) mice. Twice-daily injections of a short half-life ampakine normalized BDNF levels, activity-driven actin polymerization in dendritic spines, and LTP stabilization in 8-week-old mutants. Comparable results were obtained in 16-week-old HD mice with more severe LTP deficits. Ampakine treatments had no measurable effect on the decreased locomotor activity observed in the mutants but offset their impairments in long-term memory. Given that ampakines are well tolerated in clinical trials and were effective in this study after brief exposures, these results suggest a novel strategy for chronic treatment of the cognitive difficulties that occur in the early stages of HD.
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Manish A. Shah, Nicole A. Datson, Lakshmi Srinidhi et al. · Cell · 1993 · 3.1K citations
Liliana Menalled, Jessica D. Sison, Ioannis Dragatsis et al. · The Journal of Comparative Neurology · 2003 · 532 citations · Full text