FBXO7
F-box only protein 7 [FBX7]
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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.
MeSH Terms
- Animals
- Animals, Genetically Modified
- Carrier Proteins
- Disease Models, Animal
- Drosophila Proteins
- Drosophila melanogaster
- Eye
- F-Box Proteins
- Female
- Gene Expression
- Longevity
- Male
- Organ Specificity
- Organogenesis
- Parkinson Disease
- Protein Binding
- Protein Interaction Domains and Motifs
- alpha-Synuclein
Keywords
- Drosophila melanogaster
- FBXO7
- PI31
- Parkinson Disease
- maladie de Parkinson
- neurodegeneration
- neurodégénérescence
- nutcracker