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

Copper binding triggers compaction in N-terminal tail of human copper pump ATP7B

(2016) Tanumoy Mondol et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Enthalpy-entropy compensation at play in human copper ion transfer

(2015) Moritz S. Niemiec et al.   Scientific Reports

How copper traverses cellular membranes through the mammalian copper transporter 1, Ctr1

(2014) Helena Öhrvik et al.   Annals of the New York Academy of Sciences

Interactions between Metal-binding Domains Modulate Intracellular Targeting of Cu(I)-ATPase ATP7B, as Revealed by Nanobody Binding

(2014) Yiping Huang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

T versus D in the MTCXXC motif of copper transport proteins plays a role in directional metal transport

(2014) Moritz S. Niemiec et al.   JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY

Diverse Functional Properties of Wilson Disease ATP7B Variants

(2012) Dominik Huster et al.   GASTROENTEROLOGY

Molecular Events Initiating Exit of a Copper-transporting ATPase ATP7B from theTrans-Golgi Network

(2012) Nesrin M. Hasan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Optimization of the Additive CHARMM All-Atom Protein Force Field Targeting Improved Sampling of the Backbone ϕ, ψ and Side-Chain χ1 and χ2 Dihedral Angles

(2012) Robert B. Best et al.   Journal of Chemical Theory and Computation

In Vitro Thermodynamic Dissection of Human Copper Transfer from Chaperone to Target Protein

(2012) Moritz S. Niemiec et al.   PLoS One

Copper: An essential metal in biology

(2011) Richard A. Festa et al.   CURRENT BIOLOGY

Copper Metallochaperones

(2010) Nigel J. Robinson et al.   Annual Review of Biochemistry

Copper(I)-mediated protein–protein interactions result from suboptimal interaction surfaces

(2009) Lucia Banci et al.   BIOCHEMICAL JOURNAL

Non-linear effects of macromolecular crowding on enzymatic activity of multi-copper oxidase

(2009) Irina Pozdnyakova et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Dissecting the role of the N-terminal metal-binding domains in activating the yeast copper ATPasein vivo

(2009) Isabelle Morin et al.   FEBS Journal

Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin

(2009) Peter V. E. van den Berghe et al.   HEPATOLOGY

Interactions between Copper-binding Sites Determine the Redox Status and Conformation of the Regulatory N-terminal Domain of ATP7B

(2009) Erik S. LeShane et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

An NMR Study of the Interaction of the N-terminal Cytoplasmic Tail of the Wilson Disease Protein with Copper(I)-HAH1

(2009) Lucia Banci et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CHARMM: The biomolecular simulation program

(2009) B. R. Brooks et al.   JOURNAL OF COMPUTATIONAL CHEMISTRY

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

(2008) Lucia Banci et al.   BIOCHEMISTRY

Sequence variation in the ATP-binding domain of the Wilson disease transporter, ATP7B, affects copper transport in a yeast model system

(2008) Gloria Hsi et al.   HUMAN MUTATION

Stability and ATP Binding of the Nucleotide-binding Domain of the Wilson Disease Protein: Effect of the Common H1069Q Mutation

(2008) Agustina Rodriguez-Granillo et al.   JOURNAL OF MOLECULAR BIOLOGY

Mechanism of Cu+-transporting ATPases: Soluble Cu+ chaperones directly transfer Cu+ to transmembrane transport sites

(2008) M. Gonzalez-Guerrero et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Role of Copper in Thermal Stability of Human Ceruloplasmin

(2007) Erik Sedlák et al.   BIOPHYSICAL JOURNAL