Conformational Dynamics of Metal-Binding Domains in Wilson Disease Protein: Molecular Insights into Selective Copper Transfer†

Biochemistry ◽  
2009 ◽  
Vol 48 (25) ◽  
pp. 5849-5863 ◽  
Author(s):  
Agustina Rodriguez-Granillo ◽  
Alejandro Crespo ◽  
Pernilla Wittung-Stafshede
Keyword(s):  
Metal Binding ◽  
Wilson Disease ◽  
Conformational Dynamics ◽  
Binding Domains ◽  
Wilson Disease Protein ◽  
Disease Protein ◽  
Copper Transfer

scholarly journals Copper relay path through the N-terminus of Wilson disease protein, ATP7B

Metallomics ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1472-1480 ◽  
Author(s):  
Kumaravel Ponnandai Shanmugavel ◽  
Pernilla Wittung-Stafshede
Keyword(s):  
Metal Binding ◽  
Wilson Disease ◽  
Copper Transport ◽  
Binding Domains ◽  
Wilson Disease Protein ◽  
N Terminus ◽  
Disease Protein ◽  
Yeast Assay

Using a yeast assay, we identified the roles of ATP7B's six metal-binding domains in internal copper transport and soluble chaperone capacity.

Copper-Transfer Mechanism from the Human Chaperone Atox1 to a Metal-Binding Domain of Wilson Disease Protein

2010 ◽  
Vol 114 (10) ◽  
pp. 3698-3706 ◽  
Author(s):  
Agustina Rodriguez-Granillo ◽  
Alejandro Crespo ◽  
Dario A. Estrin ◽  
Pernilla Wittung-Stafshede
Keyword(s):  
Metal Binding ◽  
Wilson Disease ◽  
Binding Domain ◽  
Transfer Mechanism ◽  
Wilson Disease Protein ◽  
Metal Binding Domain ◽  
Disease Protein ◽  
Copper Transfer

scholarly journals Metal Binding Domains 3 and 4 of the Wilson Disease Protein: Solution Structure and Interaction with the Copper(I) Chaperone HAH1†‡

Biochemistry ◽  
2008 ◽  
Vol 47 (28) ◽  
pp. 7423-7429 ◽  
Author(s):  
Lucia Banci ◽  
Ivano Bertini ◽  
Francesca Cantini ◽  
Amy C. Rosenzweig ◽  
Liliya A. Yatsunyk
Keyword(s):  
Metal Binding ◽  
Wilson Disease ◽  
Solution Structure ◽  
Protein Solution ◽  
Binding Domains ◽  
Wilson Disease Protein ◽  
Disease Protein

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

BioMetals ◽  
2016 ◽  
Vol 30 (1) ◽  
pp. 27-35 ◽  
Author(s):  
Ranjeet Kumar ◽  
Candan Ariöz ◽  
Yaozong Li ◽  
Niklas Bosaeus ◽  
Sandra Rocha ◽  
...  
Keyword(s):  
Structural Dynamics ◽  
Metal Binding ◽  
Wilson Disease ◽  
Point Mutations ◽  
Binding Domains ◽  
Wilson Disease Protein ◽  
Disease Protein

scholarly journals Probing functional roles of Wilson disease protein (ATP7B) copper-binding domains in yeast

Metallomics ◽  
2017 ◽  
Vol 9 (7) ◽  
pp. 981-988 ◽  
Author(s):  
Kumaravel Ponnandai Shanmugavel ◽  
Dina Petranovic ◽  
Pernilla Wittung-Stafshede
Keyword(s):  
Wilson Disease ◽  
Copper Binding ◽  
Functional Roles ◽  
Binding Domains ◽  
Wilson Disease Protein ◽  
Disease Protein

A double deleted yeast system was developed to probe activity of human ATP7B variants in the presence of human Atox1.

Localization of the Wilson disease protein in murine intestine

2008 ◽  
Vol 46 (09) ◽  
Author(s):  
KH Weiss ◽  
D Gotthardt ◽  
J Wurz ◽  
U Merle ◽  
W Stremmel ◽  
...  
Keyword(s):  
Wilson Disease ◽  
Wilson Disease Protein ◽  
Disease Protein

Translocation of the Wilson disease protein ATP7B is independent of Murr1/ COMMD1 and Rab7

2008 ◽  
Vol 46 (01) ◽  
Author(s):  
KH Weiss ◽  
JC Lozoya ◽  
S Tuma ◽  
D Gotthardt ◽  
J Reichert ◽  
...  
Keyword(s):  
Wilson Disease ◽  
Wilson Disease Protein ◽  
Disease Protein
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