دورية أكاديمية
A Focus on the Beneficial Effects of Alpha Synuclein and a Re-appraisal of Synucleinopathies
العنوان: | A Focus on the Beneficial Effects of Alpha Synuclein and a Re-appraisal of Synucleinopathies |
---|---|
المؤلفون: | Larisa Ryskalin, Carla L. Busceti, Fiona Limanaqi, Francesca Biagioni, Stefano Gambardella, Francesco Fornai |
المساهمون: | L. Ryskalin, C.L. Busceti, F. Limanaqi, F. Biagioni, S. Gambardella, F. Fornai |
بيانات النشر: | Bentham Science Publishers |
سنة النشر: | 2018 |
المجموعة: | The University of Milan: Archivio Istituzionale della Ricerca (AIR) |
مصطلحات موضوعية: | Alpha synuclein, synucleinopathie, alpha synuclein aggregate, loss-of-function, co-chaperonine, neurodegeneration, neuroprotection, Settore BIO/13 - Biologia Applicata, Settore BIO/16 - Anatomia Umana |
الوصف: | Alpha synuclein (α-syn) belongs to a class of proteins which are commonly considered to play a detrimental role in neuronal survival. This assumption is based on the occurrence of a severe neuronal degeneration in patients carrying a multiplication of the α-syn gene (SNCA) and in a variety of experimental models, where overexpression of α-syn leads to cell death and neurological impairment. In these conditions, a higher amount of normally structured α-syn produces a damage, which is even worse compared with that produced by α-syn owing an abnormal structure (as occurring following point gene mutations). In line with this, knocking out the expression of α-syn is reported to protect from specific neurotoxins such as 1-methyl, 4-phenyl 1,2,3,6-tetrahydropyridine (MPTP). In the present review we briefly discuss these well-known detrimental effects but we focus on findings showing that, in specific conditions α-syn is beneficial for cell survival. This occurs during methamphetamine intoxication which is counteracted by endogenous α-syn. Similarly, the dysfunction of the chaperone cysteine-string protein-alpha leads to cell pathology which is counteracted by over-expressing α-syn. In line with this, an increased expression of α-syn protects against oxidative damage produced by dopamine. Remarkably, when the lack of α-syn is combined with a depletion of β- and γ- synucleins, alterations in brain structure and function occur. This review tries to balance the evidence showing a beneficial effect with the bulk of data reporting a detrimental effect of endogenous α-syn. The specific role of α-syn as a chaperone protein is discussed to explain such a dual effect. |
نوع الوثيقة: | article in journal/newspaper |
اللغة: | English |
العلاقة: | info:eu-repo/semantics/altIdentifier/pmid/29150919; info:eu-repo/semantics/altIdentifier/wos/WOS:000430104200007; volume:19; issue:6; firstpage:598; lastpage:611; numberofpages:14; journal:CURRENT PROTEIN & PEPTIDE SCIENCE; https://hdl.handle.net/2434/995038Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85046751466 |
DOI: | 10.2174/1389203718666171117110028 |
الإتاحة: | https://doi.org/10.2174/1389203718666171117110028Test https://hdl.handle.net/2434/995038Test |
حقوق: | info:eu-repo/semantics/openAccess |
رقم الانضمام: | edsbas.2D85BD06 |
قاعدة البيانات: | BASE |
DOI: | 10.2174/1389203718666171117110028 |
---|