Disease-causing point-mutations in metal-binding domains of Wilson disease protein decrease stability and increase structural dynamics

After cellular uptake, Copper (Cu) ions are transferred from the chaperone Atox1 to the Wilson disease protein (ATP7B) for incorporation into Cu-dependent enzymes in the secretory pathway. Human ATP7B is a large multi-domain membrane-spanning protein which, in contrast to homologues in other organis...

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Detalles Bibliográficos
Autores principales: Kumar, Ranjeet, Ariöz, Candan, Li, Yaozong, Bosaeus, Niklas, Rocha, Sandra, Wittung-Stafshede, Pernilla
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Netherlands 2016
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5285417/
https://www.ncbi.nlm.nih.gov/pubmed/27744583
http://dx.doi.org/10.1007/s10534-016-9976-7

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5285417/
https://www.ncbi.nlm.nih.gov/pubmed/27744583
http://dx.doi.org/10.1007/s10534-016-9976-7

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