title: Deficits of glutamate transmission in the striatum of toxic and genetic models of Huntington's disease.
abstract: Altered glutamate transmission in the striatum has been proposed to play a critical role in the pathophysiology of Huntington's disease (HD), a genetic disorder associated with impaired activity of the mitochondrial complex II (succinate dehydrogenase, SD). In the present study, we recorded spontaneous (sEPSCs) and miniature excitatory postsynaptic currents (mEPSCs) from striatal neurons of both toxic (systemic administration of 3-nitropropionic acid in rats) and genetic models of HD (R6/2 transgenic mice). In both models, we found a significant down-regulation of glutamate transmission, suggesting that reduced synaptic excitation of the input structure of the basal ganglia represents a physiological correlate of HD.
authors.affiliation: Clinica Neurologica, Dipartimento di Neuroscienze, Università Tor Vergata, Via Montpellier 1, 00133 Rome, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Rossi
authors.forename: Chiara
authors.initials: C
authors.lastname: Prosperetti
authors.forename: Barbara
authors.initials: B
authors.lastname: Picconi
authors.forename: Valentina
authors.initials: V
authors.lastname: De Chiara
authors.forename: Giorgia
authors.initials: G
authors.lastname: Mataluni
authors.forename: Giorgio
authors.initials: G
authors.lastname: Bernardi
authors.forename: Paolo
authors.initials: P
authors.lastname: Calabresi
authors.forename: Diego
authors.initials: D
authors.lastname: Centonze
mesh_terms: D000818:Animals; D003342:Corpus Striatum / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology*; D004195:Disease Models, Animal; D019706:Excitatory Postsynaptic Potentials / Q000187:drug effects / Q000528:radiation effects; D018698:Glutamic Acid / Q000378:metabolism*; D006816:Huntington Disease / Q000139:chemically induced / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology*; D066298:In Vitro Techniques; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009474:Neurons / Q000187:drug effects / Q000502:physiology; D009574:Nitro Compounds; D018408:Patch-Clamp Techniques / Q000379:methods; D011422:Propionates; D051381:Rats; D017207:Rats, Sprague-Dawley; D009435:Synaptic Transmission / Q000187:drug effects / Q000502:physiology
chemical_list: D009574:Nitro Compounds; D011422:Propionates; D018698:Glutamic Acid; C015392:3-nitropropionic acid