Aggregation of Mutant Cysteine String Protein-α via Fe-S Cluster-Binding is mitigated by Fe-chelators

Point mutations in cysteine string protein-α (CSPα) cause dominantly inherited adult-onset neuronal ceroid lipofuscinosis (ANCL) – a rapidly progressing and lethal neurodegenerative disease with no treatment. ANCL mutations are proposed to trigger CSPα aggregation/oligomerization, but the mechanism...

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Publicado no:Nat Struct Mol Biol
Main Authors: Naseri, Nima N., Ergel, Burçe, Kharel, Parinati, Na, Yoonmi, Huang, Qingqiu, Huang, Rong, Dolzhanskaya, Natalia, Burre, Jacqueline, Velinov, Milen T., Sharma, Manu
Formato: Artigo
Idioma:Inglês
Publicado em: 2020
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7021000/
https://ncbi.nlm.nih.gov/pubmed/32042150
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41594-020-0375-y
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