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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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Bibliografski detalji
Izdano u:Biometals
Glavni autori: Kumar, Ranjeet, Ariöz, Candan, Li, Yaozong, Bosaeus, Niklas, Rocha, Sandra, Wittung-Stafshede, Pernilla
Format: Artigo
Jezik:Inglês
Izdano: Springer Netherlands 2016
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5285417/
https://ncbi.nlm.nih.gov/pubmed/27744583
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s10534-016-9976-7
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