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Sigma-1 receptor ag...
Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration : involvement of calpastatin and the NF-kappa B pathway
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- Hyrskyluoto, A. (författare)
- Univ Helsinki, Biomedicum Helsinki, Inst Biomed Biochem & Dev Biol, FIN-00014 Helsinki, Finland.;Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland.
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- Pulli, I. (författare)
- Abo Akad Univ, Dept Biosci, FIN-20520 Turku, Finland.
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- Tornqvist, K. (författare)
- Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland.;Abo Akad Univ, Dept Biosci, FIN-20520 Turku, Finland.
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- Ho, T. Huu (författare)
- Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland.
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- Korhonen, L. (författare)
- Linköpings universitet,Univ Helsinki, Biomedicum Helsinki, Inst Biomed Biochem & Dev Biol, FIN-00014 Helsinki, Finland.;Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland.;Helsinki Univ Cent Hosp, Div Child Psychiat, Hus Helsinki 00029, Finland.
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- Lindholm, D. (författare)
- Univ Helsinki, Biomedicum Helsinki, Inst Biomed Biochem & Dev Biol, FIN-00014 Helsinki, Finland.;Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland.
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Univ Helsinki, Biomedicum Helsinki, Inst Biomed Biochem & Dev Biol, FIN-00014 Helsinki, Finland;Biomedicum Helsinki, Minerva Med Res Inst, Helsinki 00290, Finland. Abo Akad Univ, Dept Biosci, FIN-20520 Turku, Finland. (creator_code:org_t)
- 2013-05-23
- 2013
- Engelska.
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Ingår i: Cell Death and Disease. - : NATURE PUBLISHING GROUP. - 2041-4889. ; 4
- Relaterad länk:
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https://doi.org/10.1...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- Alterations in mitochondria and increased oxidative stress are associated with the disease progression in Huntington's disease (HD). Endoplasmic reticulum (ER) stress and oxidative damage are linked through the close communication between the ER and mitochondria. Sigma-1 receptor (Sig-1R) is a chaperone protein in the ER that is involved in ER stress regulation, but little is known about its role in HD or the mechanisms for cell protection. Here we show that the Sig-1R agonist, PRE084 increases cell survival and counteracts the deleterious effects caused by N-terminal mutant huntingtin proteins in neuronal PC6.3 cells. Particularly, PRE084 increased the levels of cellular antioxidants by activating the NF-kappa B pathway that is compromised by the expression of mutant huntingtin proteins. These results show that the Sig-1R agonist has beneficial effects in models of HD and that compounds affecting the Sig-1R may be promising targets for future drug development in HD.
Nyckelord
- Sigma-1 receptor
- NF-kappa B
- calpastatin
- oxidative stress
- HD
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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