1aw0: Difference between revisions
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'''FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES''' | {{Structure | ||
|PDB= 1aw0 |SIZE=350|CAPTION= <scene name='initialview01'>1aw0</scene> | |||
|SITE= | |||
|LIGAND= | |||
|ACTIVITY= [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] | |||
|GENE= | |||
}} | |||
'''FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES''' | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
1AW0 is a [ | 1AW0 is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1AW0 OCA]. | ||
==Reference== | ==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:[http:// | 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:[http://www.ncbi.nlm.nih.gov/pubmed/9437429 9437429] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Hydrogen/potassium-exchanging ATPase]] | [[Category: Hydrogen/potassium-exchanging ATPase]] | ||
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[[Category: hydrolase]] | [[Category: hydrolase]] | ||
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 10:02:57 2008'' | ||
Revision as of 11:03, 20 March 2008
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| Activity: | Hydrogen/potassium-exchanging ATPase, with EC number 3.6.3.10 | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
FOURTH METAL-BINDING DOMAIN OF THE MENKES COPPER-TRANSPORTING ATPASE, NMR, 20 STRUCTURES
OverviewOverview
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.
DiseaseDisease
Known diseases associated with this structure: Analgesia from kappa-opioid receptor agonist, female-specific , OMIM:[155555], Cutis laxa, neonatal OMIM:[300011], Melanoma susceptibility to OMIM:[155555], Menkes disease OMIM:[300011], Occipital horn syndrome OMIM:[300011], Oculocutaneous albinism, type II, modifier of OMIM:[155555], Skin/hair/eye pigmentation 2, blond hair/fair skin OMIM:[155555], Skin/hair/eye pigmentation 2, red hair/fair skin OMIM:[155555], UV-induced skin damage OMIM:[155555]
About this StructureAbout this Structure
1AW0 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
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
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