1q8l
From Proteopedia
(New page: 200px<br /> <applet load="1q8l" size="450" color="white" frame="true" align="right" spinBox="true" caption="1q8l" /> '''Second Metal Binding Domain of the Menkes A...) |
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'''Second Metal Binding Domain of the Menkes ATPase'''<br /> | '''Second Metal Binding Domain of the Menkes ATPase'''<br /> | ||
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==Disease== | ==Disease== | ||
| - | Known diseases associated with this structure: Analgesia from kappa-opioid receptor agonist, female-specific OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], | + | Known diseases associated with this structure: Analgesia from kappa-opioid receptor agonist, female-specific , OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], Cutis laxa, neonatal OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300011 300011]], Melanoma susceptibility to OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], Menkes disease OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300011 300011]], Occipital horn syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300011 300011]], Oculocutaneous albinism, type II, modifier of OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], Skin/hair/eye pigmentation 2, blond hair/fair skin OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], Skin/hair/eye pigmentation 2, red hair/fair skin OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]], UV-induced skin damage OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=155555 155555]] |
==About this Structure== | ==About this Structure== | ||
| - | 1Q8L is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1Q8L is a [http://en.wikipedia.org/wiki/Single_protein 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=1Q8L OCA]. |
==Reference== | ==Reference== | ||
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[[Category: metal binding protein]] | [[Category: metal binding protein]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 16:42:59 2008'' |
Revision as of 14:43, 15 February 2008
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Second Metal Binding Domain of the Menkes ATPase
Contents |
Overview
Biological utilisation of copper requires that the metal, in its ionic, forms, be meticulously transported, inserted into enzymes and regulatory, proteins, and excess be excreted. To understand the trafficking process, it is crucial that the structures of the proteins involved in the varied, processes be resolved. To investigate copper binding to a family of, structurally related copper-binding proteins, we have characterised the, second Menkes N-terminal domain (MNKr2). The structure, determined using, 1H and 15N heteronuclear NMR, of the reduced form of MNKr2 has revealed, two alpha-helices lying over a single beta-sheet and shows that the, binding site, a Cys(X)2Cys pair, is located on an exposed loop. 1H-15N, HSQC experiments demonstrate that binding of Cu(I) causes changes that are, localised to conserved residues adjacent to the metal binding site., Residues in this area are important to the delivery of copper by the, structurally related Cu(I) chaperones. Complementary site-directed, mutagenesis of the adjacent residues has been used to probe the structural, roles of conserved residues.
Disease
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 Structure
1Q8L is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structure and metal binding studies of the second copper binding domain of the Menkes ATPase., Jones CE, Daly NL, Cobine PA, Craik DJ, Dameron CT, J Struct Biol. 2003 Sep;143(3):209-18. PMID:14572476
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