1aw0

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[[Image:1aw0.gif|left|200px]]
[[Image:1aw0.gif|left|200px]]
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{{Structure
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|PDB= 1aw0 |SIZE=350|CAPTION= <scene name='initialview01'>1aw0</scene>
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The line below this paragraph, containing "STRUCTURE_1aw0", creates the "Structure Box" on the page.
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|ACTIVITY= <span class='plainlinks'>[http://en.wikipedia.org/wiki/Hydrogen/potassium-exchanging_ATPase Hydrogen/potassium-exchanging ATPase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.6.3.10 3.6.3.10] </span>
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{{STRUCTURE_1aw0| PDB=1aw0 | SCENE= }}
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1aw0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1aw0 OCA], [http://www.ebi.ac.uk/pdbsum/1aw0 PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1aw0 RCSB]</span>
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'''FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES'''
'''FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES'''
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[[Category: Fairbrother, W J.]]
[[Category: Fairbrother, W J.]]
[[Category: Gitschier, J.]]
[[Category: Gitschier, J.]]
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[[Category: copper-binding domain]]
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[[Category: Copper-binding domain]]
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[[Category: copper-transporting atpase]]
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[[Category: Copper-transporting atpase]]
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[[Category: hydrolase]]
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[[Category: Hydrolase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 10:45:41 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 18:48:33 2008''
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Revision as of 07:45, 2 May 2008

Template:STRUCTURE 1aw0

FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES


Overview

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(I)-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 beta alpha beta beta alpha beta 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(I)-bound structure. Ag(I) binds in a linear bicoordinate manner to the two Cys residues of the conserved GMTCxxC motif; Cu(I) 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.

About this Structure

1AW0 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Solution structure of the fourth metal-binding domain from the Menkes copper-transporting ATPase., Gitschier J, Moffat B, Reilly D, Wood WI, Fairbrother WJ, Nat Struct Biol. 1998 Jan;5(1):47-54. PMID:9437429 Page seeded by OCA on Fri May 2 10:45:41 2008

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