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ABSTRACT Menkes disease is an X-linked disorder in copper transport that results in death during early childhood. The solution structures of both apo and Ag(l)-bound forms of the fourth

metal-binding domain (mbd4) from the Menkes copper-transporting ATPase have been solved. The 72-residue mbd4 has a ferredoxin-like βαββαβ fold. Structural differences between the two forms

are limited to the metal-binding loop, which is disordered in the apo structure but well ordered in the Ag(l)-bound structure. Ag(l) binds in a linear bicoordinate manner to the two Cys

residues of the conserved GMTCxxC motif; Cu(l) likely coordinates in a similar manner. Menkes mbd4 is thus the first bicoordinate copper-binding protein to be characterized structurally.

Sequence comparisons with other heavy-metal-binding domains reveal a conserved hydrophobic core and metal-binding motif. Access through your institution Buy or subscribe This is a preview of

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BINDING DOMAINS AND TRANSPORT MECHANISM OF COPPER TRANSPORTING P-TYPE ATPASES Article Open access 27 March 2024 STRUCTURAL BASIS OF ION UPTAKE IN COPPER-TRANSPORTING P1B-TYPE ATPASES Article

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Scholar  Download references AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Howard Hughes Medical Institute and Departments of Medicine and Pediatrics, University of California, San

Francisco, California, 94143, USA Jane Gitschier * Department of Protein Chemistry, Genentech, Inc., South San Francisco, California, 90480, USA Barbara Moffat * Department of Cell Culture,

Genentech, Inc., South San Francisco, California, 90480, USA Dorothea Reilly * Department of Molecular Biology, Genentech, Inc., South San Francisco, California, 90480, USA William I. Wood *

Department of Protein Engineering, Genentech, Inc., South San Francisco, California, 90480, USA Wayne J. Fairbrother Authors * Jane Gitschier View author publications You can also search

for this author inPubMed Google Scholar * Barbara Moffat View author publications You can also search for this author inPubMed Google Scholar * Dorothea Reilly View author publications You

can also search for this author inPubMed Google Scholar * William I. Wood View author publications You can also search for this author inPubMed Google Scholar * Wayne J. Fairbrother View

author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Gitschier, J., Moffat,

B., Reilly, D. _et al._ Solution structure of the fourth metal-binding domain from the Menkes copper-transporting ATPase. _Nat Struct Mol Biol_ 5, 47–54 (1998).

https://doi.org/10.1038/nsb0198-47 Download citation * Received: 08 October 1997 * Accepted: 21 November 1997 * Issue Date: 01 January 1998 * DOI: https://doi.org/10.1038/nsb0198-47 SHARE

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