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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Detalles Bibliográficos
Autores principales: Naseri, Nima N., Ergel, Burçe, Kharel, Parinati, Na, Yoonmi, Huang, Qingqiu, Huang, Rong, Dolzhanskaya, Natalia, Burre, Jacqueline, Velinov, Milen T., Sharma, Manu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7021000/
https://www.ncbi.nlm.nih.gov/pubmed/32042150
http://dx.doi.org/10.1038/s41594-020-0375-y

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7021000/
https://www.ncbi.nlm.nih.gov/pubmed/32042150
http://dx.doi.org/10.1038/s41594-020-0375-y

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