The FBXO7 homologue nutcracker and binding partner PI31 in Drosophila melanogaster models of Parkinson's disease.
Identifieur interne : 000017 ( Main/Exploration ); précédent : 000016; suivant : 000018The FBXO7 homologue nutcracker and binding partner PI31 in Drosophila melanogaster models of Parkinson's disease.
Auteurs : Eric M. Merzetti [Canada] ; Lindsay A. Dolomount [Canada] ; Brian E. Staveley [Canada]Source :
- Genome [ 1480-3321 ] ; 2017.
English descriptors
- KwdEn :
- Animals, Animals, Genetically Modified, Carrier Proteins (chemistry), Carrier Proteins (genetics), Carrier Proteins (metabolism), Disease Models, Animal, Drosophila Proteins (chemistry), Drosophila Proteins (genetics), Drosophila Proteins (metabolism), Drosophila melanogaster (genetics), Drosophila melanogaster (metabolism), Eye (embryology), Eye (metabolism), F-Box Proteins (chemistry), F-Box Proteins (genetics), F-Box Proteins (metabolism), Female, Gene Expression, Longevity (genetics), Male, Organ Specificity (genetics), Organogenesis (genetics), Parkinson Disease (genetics), Parkinson Disease (metabolism), Protein Binding, Protein Interaction Domains and Motifs, alpha-Synuclein (genetics), alpha-Synuclein (metabolism).
- MESH :
- chemical , chemistry : Carrier Proteins, Drosophila Proteins, F-Box Proteins.
- chemical , genetics : Carrier Proteins, Drosophila Proteins, F-Box Proteins, alpha-Synuclein.
- chemical , metabolism : Carrier Proteins, Drosophila Proteins, F-Box Proteins, alpha-Synuclein.
- embryology : Eye.
- genetics : Drosophila melanogaster, Longevity, Organ Specificity, Organogenesis, Parkinson Disease.
- metabolism : Drosophila melanogaster, Eye, Parkinson Disease.
- Animals, Animals, Genetically Modified, Disease Models, Animal, Female, Gene Expression, Male, Protein Binding, Protein Interaction Domains and Motifs.
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.
DOI: 10.1139/gen-2016-0087
PubMed: 27936908
Affiliations:
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Le document en format XML
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Dolomount</name><affiliation wicri:level="1"><nlm:affiliation>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9</wicri:regionArea><wicri:noRegion>NL A1B 3X9</wicri:noRegion></affiliation></author><author><name sortKey="Staveley, Brian E" sort="Staveley, Brian E" uniqKey="Staveley B" first="Brian E" last="Staveley">Brian E. 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Merzetti</name><affiliation wicri:level="1"><nlm:affiliation>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9</wicri:regionArea><wicri:noRegion>NL A1B 3X9</wicri:noRegion></affiliation></author><author><name sortKey="Dolomount, Lindsay A" sort="Dolomount, Lindsay A" uniqKey="Dolomount L" first="Lindsay A" last="Dolomount">Lindsay A. Dolomount</name><affiliation wicri:level="1"><nlm:affiliation>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9</wicri:regionArea><wicri:noRegion>NL A1B 3X9</wicri:noRegion></affiliation></author><author><name sortKey="Staveley, Brian E" sort="Staveley, Brian E" uniqKey="Staveley B" first="Brian E" last="Staveley">Brian E. Staveley</name><affiliation wicri:level="1"><nlm:affiliation>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Department of Biology, Memorial University of Newfoundland, 232 Elizabeth Avenue, St. John's, NL A1B 3X9</wicri:regionArea><wicri:noRegion>NL A1B 3X9</wicri:noRegion></affiliation></author></analytic><series><title level="j">Genome</title><idno type="eISSN">1480-3321</idno><imprint><date when="2017" type="published">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Animals</term><term>Animals, Genetically Modified</term><term>Carrier Proteins (chemistry)</term><term>Carrier Proteins (genetics)</term><term>Carrier Proteins (metabolism)</term><term>Disease Models, Animal</term><term>Drosophila Proteins (chemistry)</term><term>Drosophila Proteins (genetics)</term><term>Drosophila Proteins (metabolism)</term><term>Drosophila melanogaster (genetics)</term><term>Drosophila melanogaster (metabolism)</term><term>Eye (embryology)</term><term>Eye (metabolism)</term><term>F-Box Proteins (chemistry)</term><term>F-Box Proteins (genetics)</term><term>F-Box Proteins (metabolism)</term><term>Female</term><term>Gene Expression</term><term>Longevity (genetics)</term><term>Male</term><term>Organ Specificity (genetics)</term><term>Organogenesis (genetics)</term><term>Parkinson Disease (genetics)</term><term>Parkinson Disease (metabolism)</term><term>Protein Binding</term><term>Protein Interaction Domains and Motifs</term><term>alpha-Synuclein (genetics)</term><term>alpha-Synuclein (metabolism)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="chemistry" xml:lang="en"><term>Carrier Proteins</term><term>Drosophila Proteins</term><term>F-Box Proteins</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Carrier Proteins</term><term>Drosophila Proteins</term><term>F-Box Proteins</term><term>alpha-Synuclein</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Carrier Proteins</term><term>Drosophila Proteins</term><term>F-Box Proteins</term><term>alpha-Synuclein</term></keywords><keywords scheme="MESH" qualifier="embryology" xml:lang="en"><term>Eye</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Drosophila melanogaster</term><term>Longevity</term><term>Organ Specificity</term><term>Organogenesis</term><term>Parkinson Disease</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Drosophila melanogaster</term><term>Eye</term><term>Parkinson Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Animals</term><term>Animals, Genetically Modified</term><term>Disease Models, Animal</term><term>Female</term><term>Gene Expression</term><term>Male</term><term>Protein Binding</term><term>Protein Interaction Domains and Motifs</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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.</div></front></TEI><affiliations><list><country><li>Canada</li></country></list><tree><country name="Canada"><noRegion><name sortKey="Merzetti, Eric M" sort="Merzetti, Eric M" uniqKey="Merzetti E" first="Eric M" last="Merzetti">Eric M. Merzetti</name></noRegion><name sortKey="Dolomount, Lindsay A" sort="Dolomount, Lindsay A" uniqKey="Dolomount L" first="Lindsay A" last="Dolomount">Lindsay A. Dolomount</name><name sortKey="Staveley, Brian E" sort="Staveley, Brian E" uniqKey="Staveley B" first="Brian E" last="Staveley">Brian E. Staveley</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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