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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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| Publié dans: | Biometals |
|---|---|
| Auteurs principaux: | , , , , , |
| Format: | Artigo |
| Langue: | Inglês |
| Publié: |
Springer Netherlands
2016
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| Sujets: | |
| Accès en ligne: | 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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