title: The FBXO7 homologue nutcracker and binding partner PI31 in Drosophila melanogaster models of Parkinson's disease.
abstract: Parkinsonian-pyramidal syndrome (PPS) is an early onset form of Parkinson's disease (PD) that shows degeneration of the extrapyramidal region of the brain to result in a severe form of PD. The toxic protein build-up has been implicated in the onset of PPS. Protein removal is mediated by an intracellular proteasome complex: an E3 ubiquitin ligase, the targeting component, is essential for function. FBXO7 encodes the F-box component of the SCF E3 ubiquitin ligase linked to familial forms of PPS. The Drosophila melanogaster homologue nutcracker (ntc) and a binding partner, PI31, have been shown to be active in proteasome function. We show that altered expression of either ntc or PI31 in dopaminergic neurons leads to a decrease in longevity and locomotor ability, phenotypes both associated with models of PD. Furthermore, expression of ntc-RNAi in an established α-synuclein-dependent model of PD rescues the phenotypes of diminished longevity and locomotor control.
authors.affiliation: Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.|Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.
authors.forename: Eric M
authors.initials: EM
authors.lastname: Merzetti
authors.affiliation: Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.|Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.
authors.forename: Lindsay A
authors.initials: LA
authors.lastname: Dolomount
authors.affiliation: Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.|Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.
authors.forename: Brian E
authors.initials: BE
authors.lastname: Staveley
mesh_terms: D000818:Animals; D030801:Animals, Genetically Modified; D002352:Carrier Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D004195:Disease Models, Animal; D029721:Drosophila Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D004331:Drosophila melanogaster / Q000235:genetics / Q000378:metabolism*; D005123:Eye / Q000196:embryology / Q000378:metabolism; D044783:F-Box Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D005260:Female; D015870:Gene Expression; D008136:Longevity / Q000235:genetics; D008297:Male; D009928:Organ Specificity / Q000235:genetics; D038081:Organogenesis / Q000235:genetics; D010300:Parkinson Disease / Q000235:genetics / Q000378:metabolism*; D011485:Protein Binding; D054730:Protein Interaction Domains and Motifs; D051844:alpha-Synuclein / Q000235:genetics / Q000378:metabolism
chemical_list: D002352:Carrier Proteins; D029721:Drosophila Proteins; D044783:F-Box Proteins; C558902:PI31 protein, Drosophila; D051844:alpha-Synuclein
keywords: Drosophila melanogaster; FBXO7; PI31; Parkinson Disease; maladie de Parkinson; neurodegeneration; neurodégénérescence; nutcracker