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A molecular chaperone activity of CCS restores the maturation of SOD1 fALS mutants

Superoxide dismutase 1 (SOD1) is an important metalloprotein for cellular oxidative stress defence, that is mutated in familiar variants of Amyotrophic Lateral Sclerosis (fALS). Some mutations destabilize the apo protein, leading to the formation of misfolded, toxic species. The Copper Chaperone for...

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Publicat a:Sci Rep
Autors principals: Luchinat, Enrico, Barbieri, Letizia, Banci, Lucia
Format: Artigo
Idioma:Inglês
Publicat: Nature Publishing Group UK 2017
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC5727297/
https://ncbi.nlm.nih.gov/pubmed/29234142
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-017-17815-y
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