title: Aberrant striatal synaptic plasticity in monogenic parkinsonisms.
abstract: In the recent past, the pathogenesis of Parkinson's disease (PD) has evolved from a neurodegenerative disorder considered entirely sporadic to a disease with an unequivocal genetic component. Indeed, different inherited forms of PD have been discovered and characterized, although the functional roles of the gene products identified are still under intense investigation. To gain a better understanding of the cellular and molecular pathogenic mechanisms of hereditary forms of PD, different animal models have been generated. Although most of the rodent models display neither obvious behavioral impairment nor evidence for neurodegeneration, remarkable abnormalities of dopamine-mediated neurotransmission and corticostriatal synaptic plasticity have been described, indicative of a fundamental distortion of network function within the basal ganglia. The picture emerging from a critical review of recent data on monogenic parkinsonisms suggests that mutations in PD genes might cause developmental rearrangements in the corticobasal ganglia circuitry, compensating the dopaminergic dysfunction observed both in mice and humans, in order to maintain proper motor function.
authors.affiliation: Department of Neuroscience, University of Rome Tor Vergata, Rome, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Madeo
authors.forename: G
authors.initials: G
authors.lastname: Martella
authors.forename: T
authors.initials: T
authors.lastname: Schirinzi
authors.forename: G
authors.initials: G
authors.lastname: Ponterio
authors.forename: J
authors.initials: J
authors.lastname: Shen
authors.forename: P
authors.initials: P
authors.lastname: Bonsi
authors.forename: A
authors.initials: A
authors.lastname: Pisani
mesh_terms: D000818:Animals; D003342:Corpus Striatum / Q000503:physiopathology*; D004195:Disease Models, Animal; D059290:Dopaminergic Neurons / Q000502:physiology*; D006706:Homeostasis / Q000235:genetics / Q000502:physiology; D008959:Models, Neurological; D009434:Neural Pathways / Q000503:physiopathology; D009473:Neuronal Plasticity / Q000235:genetics / Q000502:physiology*; D020734:Parkinsonian Disorders / Q000235:genetics* / Q000503:physiopathology*; D009435:Synaptic Transmission / Q000235:genetics* / Q000502:physiology