Synphilin-1 attenuates neuronal degeneration in the A53T -synuclein transgenic mouse model
Identifieur interne : 000431 ( Main/Exploration ); précédent : 000430; suivant : 000432Synphilin-1 attenuates neuronal degeneration in the A53T -synuclein transgenic mouse model
Auteurs : Wanli W. Smith [États-Unis] ; Zhaohui Liu ; Yideng Liang ; Naoki Masuda ; Debbie A. Swing ; Nancy A. Jenkins ; Neal G. Copeland ; Juan C. Troncoso ; Mikhail Pletnikov ; Ted M. Dawson ; Lee J. Martin ; Timothy H. Moran ; Michael K. Lee ; David R. Borchelt ; Christopher A. Ross [États-Unis]Source :
- Human Molecular Genetics [ 0964-6906 ] ; 2010-06-01.
Abstract
Genetic alterations in -synuclein cause autosomal dominant familial Parkinsonism and may contribute to sporadic Parkinson's disease (PD). Synphilin-1 is an -synuclein-interacting protein, with implications in PD pathogenesis related to protein aggregation. Currently, the in vivo role of synphilin-1 in -synuclein-linked pathogenesis is not fully understood. Using the mouse prion protein promoter, we generated synphilin-1 transgenic mice, which did not display PD-like phenotypes. However, synphilin-1/A53T -synuclein double-transgenic mice survived longer than A53T -synuclein single-transgenic mice. There were attenuated A53T -synuclein-induced motor abnormalities and decreased astroglial reaction and neuronal degeneration in brains in double-transgenic mice. Overexpression of synphilin-1 decreased caspase-3 activation, increased beclin-1 and LC3 II expression and promoted formation of aggresome-like structures, suggesting that synphilin-1 alters multiple cellular pathways to protect against neuronal degeneration. These studies demonstrate that synphilin-1 can diminish the severity of -synucleinopathy and play a neuroprotective role against A53T -synuclein toxicity in vivo.
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DOI: 10.1093/hmg/ddq086
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Synphilin-1 is an -synuclein-interacting protein, with implications in PD pathogenesis related to protein aggregation. Currently, the in vivo role of synphilin-1 in -synuclein-linked pathogenesis is not fully understood. Using the mouse prion protein promoter, we generated synphilin-1 transgenic mice, which did not display PD-like phenotypes. However, synphilin-1/A53T -synuclein double-transgenic mice survived longer than A53T -synuclein single-transgenic mice. There were attenuated A53T -synuclein-induced motor abnormalities and decreased astroglial reaction and neuronal degeneration in brains in double-transgenic mice. Overexpression of synphilin-1 decreased caspase-3 activation, increased beclin-1 and LC3 II expression and promoted formation of aggresome-like structures, suggesting that synphilin-1 alters multiple cellular pathways to protect against neuronal degeneration. These studies demonstrate that synphilin-1 can diminish the severity of -synucleinopathy and play a neuroprotective role against A53T -synuclein toxicity in vivo.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country></list><tree><noCountry><name sortKey="Borchelt, David R" sort="Borchelt, David R" uniqKey="Borchelt D" first="David R." last="Borchelt">David R. Borchelt</name><name sortKey="Copeland, Neal G" sort="Copeland, Neal G" uniqKey="Copeland N" first="Neal G." last="Copeland">Neal G. Copeland</name><name sortKey="Dawson, Ted M" sort="Dawson, Ted M" uniqKey="Dawson T" first="Ted M." last="Dawson">Ted M. Dawson</name><name sortKey="Jenkins, Nancy A" sort="Jenkins, Nancy A" uniqKey="Jenkins N" first="Nancy A." last="Jenkins">Nancy A. Jenkins</name><name sortKey="Lee, Michael K" sort="Lee, Michael K" uniqKey="Lee M" first="Michael K." last="Lee">Michael K. Lee</name><name sortKey="Liang, Yideng" sort="Liang, Yideng" uniqKey="Liang Y" first="Yideng" last="Liang">Yideng Liang</name><name sortKey="Liu, Zhaohui" sort="Liu, Zhaohui" uniqKey="Liu Z" first="Zhaohui" last="Liu">Zhaohui Liu</name><name sortKey="Martin, Lee J" sort="Martin, Lee J" uniqKey="Martin L" first="Lee J." last="Martin">Lee J. Martin</name><name sortKey="Masuda, Naoki" sort="Masuda, Naoki" uniqKey="Masuda N" first="Naoki" last="Masuda">Naoki Masuda</name><name sortKey="Moran, Timothy H" sort="Moran, Timothy H" uniqKey="Moran T" first="Timothy H." last="Moran">Timothy H. Moran</name><name sortKey="Pletnikov, Mikhail" sort="Pletnikov, Mikhail" uniqKey="Pletnikov M" first="Mikhail" last="Pletnikov">Mikhail Pletnikov</name><name sortKey="Swing, Debbie A" sort="Swing, Debbie A" uniqKey="Swing D" first="Debbie A." last="Swing">Debbie A. Swing</name><name sortKey="Troncoso, Juan C" sort="Troncoso, Juan C" uniqKey="Troncoso J" first="Juan C." last="Troncoso">Juan C. Troncoso</name></noCountry><country name="États-Unis"><noRegion><name sortKey="Smith, Wanli W" sort="Smith, Wanli W" uniqKey="Smith W" first="Wanli W." last="Smith">Wanli W. Smith</name></noRegion><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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