An αB-Crystallin Peptide Rescues Compartmentalization and Trafficking Response to Cu Overload of ATP7B-H1069Q, the Most Frequent Cause of Wilson Disease in the Caucasian Population

The H1069Q substitution is the most frequent mutation of the Cu transporter ATP7B that causes Wilson disease in the Caucasian population. ATP7B localizes to the Golgi complex in hepatocytes, but, in the presence of excessive Cu, it relocates to the endo-lysosomal compartment to excrete Cu via bile c...

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Detalles Bibliográficos
Autores principales: Allocca, Simona, Ciano, Michela, Ciardulli, Maria Camilla, D’Ambrosio, Chiara, Scaloni, Andrea, Sarnataro, Daniela, Caporaso, Maria Gabriella, D’Agostino, Massimo, Bonatti, Stefano
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6073935/
https://www.ncbi.nlm.nih.gov/pubmed/29954118
http://dx.doi.org/10.3390/ijms19071892

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6073935/
https://www.ncbi.nlm.nih.gov/pubmed/29954118
http://dx.doi.org/10.3390/ijms19071892

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